Amy Rothman Schonfeld

Major finding: Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduced risk (HR 0.83, 95% CI 0.44–1.57) of the primary composite CV end point of myocardial infarction, stroke, and CV-related death in patients with rheumatoid arthritis. Patients using a TNF inhibitor had a 61% reduced risk (HR 0.39, 95% CI 0.18–0.74) of the end point.

Data source: Longitudinal cohort study of the CORRONA registry.

Disclosures: Dr. Greenberg has done consulting for Centocor, CORRONA, Novartis Pharmaceuticals, Roche Laboratories, and UCB. He has received research and grant support from the Arthritis Foundation and the National Institutes of Health. He receives consulting fees from, or owns stock shares in, AstraZeneca, CORRONA, Genentech, Novartis, and Pfizer.

NEW YORK – Tumor necrosis factor inhibitors reduced the risk of myocardial infarction, stroke, and cardiovascular-related disease in patients with rheumatoid arthritis, according to Dr. Jeffrey D. Greenberg, who presented the findings at the meeting. Methotrexate was not associated with a reduced risk of cardiovascular events, and prednisone use was associated with a dose-dependent increased risk.

The findings come from CORRONA (Consortium of Rheumatology Researchers of North America), a registry of patients with RA or psoriatic arthritis. The study population involved 10,156 patients followed for a median of 23 months. A subset analysis was performed and 88 patients were identified who had experienced nonfatal myocardial infarction (n = 26), transient ischemic attack or stroke (n = 45), or CV-related death (n = 17) (Ann. Rheum. Dis. 2011;70:576–82).

In an editorial accompanying Dr. Greenberg's report (Ann. Rheum. Dis. 2011 70:561–2), Dr. Johan Askling of Karolinska University Hospital, Stockholm, and Dr. Will Dixon of the University of Manchester (England) praised the study's methodology and transparency. But they noted that other studies, like those published by the British Society for Rheumatology Biologics Register (BSRBR), do not support a CV protective effect for TNF inhibitors.

Nonbiologic disease-modifying antirheumatic drugs (DMARDs) other than methotrexate served as the reference cohort for all comparisons. Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduction in risk (hazard ratio 0.83, 95% confidence interval C0.44–1.57). In contrast, patients using a TNF inhibitor had a 61% lower risk of the primary composite CV end point (HR 0.39, 95% CI 0.18–0.74).

Dr. Greenberg also showed significant reductions in risk for those taking TNF inhibitors compared with those taking nonbiological DMARDs, as measured by a composite end point that included CV deaths (HR 0.39, 95% CI 0.19–0.82) and a composite end point that excluded CV deaths (HR 0.35, 95% CI 0.16–0.74), and nonfatal MI (HR 0.24, 95% CI 0.06–0.95). There was a trend toward reduced risk for nonfatal TIA/stroke (HR 0.44, 95% CI 0.18–1.09). “These data indicate that TNF antagonists may represent a therapeutic strategy to attenuate the heightened CV risk experienced by RA patients,” Dr. Greenberg said.

“As expected, there was a dose-dependent increased risk of CV events with steroids,” said Dr. Greenberg of New York University. Prednisone dosesbof 1–2.5 mg and 3–7 mg almost doubled the risk (HR 1.90 and 1.89, respectively) while doses greater than 7 mg tripled the risk (HR 3.00, 95% CI 1.44–6.25) (p = 0.04).

Dr. Greenberg advocates a three-step strategy to decrease CV risk in RA. The first is to adhere to recommended CV disease prevention, screening, and guidelines. This includes management of “traditional” CV risk factors such as blood pressure, diabetes, lipids, smoking, and obesity, he said. The second component – as supported by his study's results – is to minimize use of NSAIDs and steroids, and to utilize steroid-sparing options if possible. The third component is to aim for tighter control of RA disease activity.

For patients at increased CV risk, “I would treat RA aggressively,” said Dr. Greenberg. Even so, he acknowledged that it has not been proved that the strategy would alter the natural course of developing CV disease. Measures that should be targeted might include a specific disease activity score, C-reactive protein levels, or other inflammatory biomarkers, he said.

Major finding: Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduced risk (HR 0.83, 95% CI 0.44–1.57) of the primary composite CV end point of myocardial infarction, stroke, and CV-related death in patients with rheumatoid arthritis. Patients using a TNF inhibitor had a 61% reduced risk (HR 0.39, 95% CI 0.18–0.74) of the end point.

Data source: Longitudinal cohort study of the CORRONA registry.

Disclosures: Dr. Greenberg has done consulting for Centocor, CORRONA, Novartis Pharmaceuticals, Roche Laboratories, and UCB. He has received research and grant support from the Arthritis Foundation and the National Institutes of Health. He receives consulting fees from, or owns stock shares in, AstraZeneca, CORRONA, Genentech, Novartis, and Pfizer.

NEW YORK – Tumor necrosis factor inhibitors reduced the risk of myocardial infarction, stroke, and cardiovascular-related disease in patients with rheumatoid arthritis, according to Dr. Jeffrey D. Greenberg, who presented the findings at the meeting. Methotrexate was not associated with a reduced risk of cardiovascular events, and prednisone use was associated with a dose-dependent increased risk.

The findings come from CORRONA (Consortium of Rheumatology Researchers of North America), a registry of patients with RA or psoriatic arthritis. The study population involved 10,156 patients followed for a median of 23 months. A subset analysis was performed and 88 patients were identified who had experienced nonfatal myocardial infarction (n = 26), transient ischemic attack or stroke (n = 45), or CV-related death (n = 17) (Ann. Rheum. Dis. 2011;70:576–82).

In an editorial accompanying Dr. Greenberg's report (Ann. Rheum. Dis. 2011 70:561–2), Dr. Johan Askling of Karolinska University Hospital, Stockholm, and Dr. Will Dixon of the University of Manchester (England) praised the study's methodology and transparency. But they noted that other studies, like those published by the British Society for Rheumatology Biologics Register (BSRBR), do not support a CV protective effect for TNF inhibitors.

Nonbiologic disease-modifying antirheumatic drugs (DMARDs) other than methotrexate served as the reference cohort for all comparisons. Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduction in risk (hazard ratio 0.83, 95% confidence interval C0.44–1.57). In contrast, patients using a TNF inhibitor had a 61% lower risk of the primary composite CV end point (HR 0.39, 95% CI 0.18–0.74).

Dr. Greenberg also showed significant reductions in risk for those taking TNF inhibitors compared with those taking nonbiological DMARDs, as measured by a composite end point that included CV deaths (HR 0.39, 95% CI 0.19–0.82) and a composite end point that excluded CV deaths (HR 0.35, 95% CI 0.16–0.74), and nonfatal MI (HR 0.24, 95% CI 0.06–0.95). There was a trend toward reduced risk for nonfatal TIA/stroke (HR 0.44, 95% CI 0.18–1.09). “These data indicate that TNF antagonists may represent a therapeutic strategy to attenuate the heightened CV risk experienced by RA patients,” Dr. Greenberg said.

“As expected, there was a dose-dependent increased risk of CV events with steroids,” said Dr. Greenberg of New York University. Prednisone dosesbof 1–2.5 mg and 3–7 mg almost doubled the risk (HR 1.90 and 1.89, respectively) while doses greater than 7 mg tripled the risk (HR 3.00, 95% CI 1.44–6.25) (p = 0.04).

Dr. Greenberg advocates a three-step strategy to decrease CV risk in RA. The first is to adhere to recommended CV disease prevention, screening, and guidelines. This includes management of “traditional” CV risk factors such as blood pressure, diabetes, lipids, smoking, and obesity, he said. The second component – as supported by his study's results – is to minimize use of NSAIDs and steroids, and to utilize steroid-sparing options if possible. The third component is to aim for tighter control of RA disease activity.

For patients at increased CV risk, “I would treat RA aggressively,” said Dr. Greenberg. Even so, he acknowledged that it has not been proved that the strategy would alter the natural course of developing CV disease. Measures that should be targeted might include a specific disease activity score, C-reactive protein levels, or other inflammatory biomarkers, he said.

Major finding: Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduced risk (HR 0.83, 95% CI 0.44–1.57) of the primary composite CV end point of myocardial infarction, stroke, and CV-related death in patients with rheumatoid arthritis. Patients using a TNF inhibitor had a 61% reduced risk (HR 0.39, 95% CI 0.18–0.74) of the end point.

Data source: Longitudinal cohort study of the CORRONA registry.

Disclosures: Dr. Greenberg has done consulting for Centocor, CORRONA, Novartis Pharmaceuticals, Roche Laboratories, and UCB. He has received research and grant support from the Arthritis Foundation and the National Institutes of Health. He receives consulting fees from, or owns stock shares in, AstraZeneca, CORRONA, Genentech, Novartis, and Pfizer.

NEW YORK – Tumor necrosis factor inhibitors reduced the risk of myocardial infarction, stroke, and cardiovascular-related disease in patients with rheumatoid arthritis, according to Dr. Jeffrey D. Greenberg, who presented the findings at the meeting. Methotrexate was not associated with a reduced risk of cardiovascular events, and prednisone use was associated with a dose-dependent increased risk.

The findings come from CORRONA (Consortium of Rheumatology Researchers of North America), a registry of patients with RA or psoriatic arthritis. The study population involved 10,156 patients followed for a median of 23 months. A subset analysis was performed and 88 patients were identified who had experienced nonfatal myocardial infarction (n = 26), transient ischemic attack or stroke (n = 45), or CV-related death (n = 17) (Ann. Rheum. Dis. 2011;70:576–82).

In an editorial accompanying Dr. Greenberg's report (Ann. Rheum. Dis. 2011 70:561–2), Dr. Johan Askling of Karolinska University Hospital, Stockholm, and Dr. Will Dixon of the University of Manchester (England) praised the study's methodology and transparency. But they noted that other studies, like those published by the British Society for Rheumatology Biologics Register (BSRBR), do not support a CV protective effect for TNF inhibitors.

Nonbiologic disease-modifying antirheumatic drugs (DMARDs) other than methotrexate served as the reference cohort for all comparisons. Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduction in risk (hazard ratio 0.83, 95% confidence interval C0.44–1.57). In contrast, patients using a TNF inhibitor had a 61% lower risk of the primary composite CV end point (HR 0.39, 95% CI 0.18–0.74).

Dr. Greenberg also showed significant reductions in risk for those taking TNF inhibitors compared with those taking nonbiological DMARDs, as measured by a composite end point that included CV deaths (HR 0.39, 95% CI 0.19–0.82) and a composite end point that excluded CV deaths (HR 0.35, 95% CI 0.16–0.74), and nonfatal MI (HR 0.24, 95% CI 0.06–0.95). There was a trend toward reduced risk for nonfatal TIA/stroke (HR 0.44, 95% CI 0.18–1.09). “These data indicate that TNF antagonists may represent a therapeutic strategy to attenuate the heightened CV risk experienced by RA patients,” Dr. Greenberg said.

“As expected, there was a dose-dependent increased risk of CV events with steroids,” said Dr. Greenberg of New York University. Prednisone dosesbof 1–2.5 mg and 3–7 mg almost doubled the risk (HR 1.90 and 1.89, respectively) while doses greater than 7 mg tripled the risk (HR 3.00, 95% CI 1.44–6.25) (p = 0.04).

Dr. Greenberg advocates a three-step strategy to decrease CV risk in RA. The first is to adhere to recommended CV disease prevention, screening, and guidelines. This includes management of “traditional” CV risk factors such as blood pressure, diabetes, lipids, smoking, and obesity, he said. The second component – as supported by his study's results – is to minimize use of NSAIDs and steroids, and to utilize steroid-sparing options if possible. The third component is to aim for tighter control of RA disease activity.

For patients at increased CV risk, “I would treat RA aggressively,” said Dr. Greenberg. Even so, he acknowledged that it has not been proved that the strategy would alter the natural course of developing CV disease. Measures that should be targeted might include a specific disease activity score, C-reactive protein levels, or other inflammatory biomarkers, he said.

NEW YORK – Imaging seems to be the sine qua non of determining cardiovascular disease risk in patients with rheumatoid arthritis.

Dr. Jeffrey D. Greenberg noted that, over the last 10–15 years, epidemiologic studies have shown patients with rheumatoid arthritis (RA) have a twofold increase in the risk of myocardial infarction and stroke and an increase in cardiovascular-related deaths.

“An important issue we face is how can we risk stratify our patients to predict who will develop cardiovascular disease? Imaging is a promising area that may help us develop biomarkers of risk or better understand pathophysiological mechanisms of RA,” said Dr. Greenberg, who is director of the Arthritis Translational Registry and Biorepository at NYU Hospital for Joint Diseases.

The need for precise tools with which to predict risk has become more urgent with the recently published findings that carotid ultrasound measurement of carotid intima-media thickness has been found to predict coronary events in patients with RA, independent of traditional cardiovascular risk factors and manifestations of RA.

The study, conducted by Dr. Matthew R. Evans and his associates at Brooke Army Medical Center, Fort Sam Houston, Tex., found that there appears to be a dose-dependent relationship between plaque and risk, with a 2.5-fold increase with unilateral plaque and 4.3-fold increase with bilateral carotid plaque, suggesting that atherosclerosis plays a significant role in acute coronary events in patients with RA (Arthritis Rheum. 2011 [doi:10.1002/art.30265]).

In discussing Dr. Evans's research at his presentation at the meeting, Dr. Greenberg said that this is the first study to demonstrate the predictive value of measuring carotid intima-media thickness and plaque for cardiovascular events in RA patients.

In the Evans study, carotid ultrasounds were performed on 636 RA patients as part of the prospective ORALE (Outcome of Rheumatoid Arthritis Longitudinal Evaluation). These patients were followed for 3,402 person-years and in that time 84 patients experienced 121 new or recurrent acute coronary syndrome (ACS) events, such as myocardial infarction, unstable angina, cardiac arrest, or death from ischemic heart disease. The rate of ACS events was 3.5/100 patient-years for this group. If only those without a prior history of ACS were analyzed, this group had 66 ACS events, with an incidence of 2.1 ACS/100 person-years.

Multivariate analysis of baseline factors associated with incident or recurrent acute coronary syndromes revealed that two markers were independent predictors of a subsequent coronary event. Having a past cardiovascular event raised the risk almost threefold (hazard ratio, 2.87) and carotid intima-media thickness also raised the risk significantly (HR, 1.61). After substituting carotid plaque for intima-media thickness, the investigators found a 2.5-fold increase in risk for unilateral plaque and almost a 6-fold increase in risk for bilateral plaque.

The findings confirmed that traditional demographic and cardiovascular risk factors also predict coronary events as would be expected. These include male gender (HR, 1.94), diabetes (HR, 2.24), and hypertension (HR, 1.56). Measures of RA severity, such as swollen joint counts (HR, 1.03) and cumulative prednisone dose of 20 g (HR, 2.12) also had predictive value.

Dr. Greenberg receives consulting fees from Genentech Inc.

NEW YORK – Imaging seems to be the sine qua non of determining cardiovascular disease risk in patients with rheumatoid arthritis.

Dr. Jeffrey D. Greenberg noted that, over the last 10–15 years, epidemiologic studies have shown patients with rheumatoid arthritis (RA) have a twofold increase in the risk of myocardial infarction and stroke and an increase in cardiovascular-related deaths.

“An important issue we face is how can we risk stratify our patients to predict who will develop cardiovascular disease? Imaging is a promising area that may help us develop biomarkers of risk or better understand pathophysiological mechanisms of RA,” said Dr. Greenberg, who is director of the Arthritis Translational Registry and Biorepository at NYU Hospital for Joint Diseases.

The need for precise tools with which to predict risk has become more urgent with the recently published findings that carotid ultrasound measurement of carotid intima-media thickness has been found to predict coronary events in patients with RA, independent of traditional cardiovascular risk factors and manifestations of RA.

The study, conducted by Dr. Matthew R. Evans and his associates at Brooke Army Medical Center, Fort Sam Houston, Tex., found that there appears to be a dose-dependent relationship between plaque and risk, with a 2.5-fold increase with unilateral plaque and 4.3-fold increase with bilateral carotid plaque, suggesting that atherosclerosis plays a significant role in acute coronary events in patients with RA (Arthritis Rheum. 2011 [doi:10.1002/art.30265]).

In discussing Dr. Evans's research at his presentation at the meeting, Dr. Greenberg said that this is the first study to demonstrate the predictive value of measuring carotid intima-media thickness and plaque for cardiovascular events in RA patients.

In the Evans study, carotid ultrasounds were performed on 636 RA patients as part of the prospective ORALE (Outcome of Rheumatoid Arthritis Longitudinal Evaluation). These patients were followed for 3,402 person-years and in that time 84 patients experienced 121 new or recurrent acute coronary syndrome (ACS) events, such as myocardial infarction, unstable angina, cardiac arrest, or death from ischemic heart disease. The rate of ACS events was 3.5/100 patient-years for this group. If only those without a prior history of ACS were analyzed, this group had 66 ACS events, with an incidence of 2.1 ACS/100 person-years.

Multivariate analysis of baseline factors associated with incident or recurrent acute coronary syndromes revealed that two markers were independent predictors of a subsequent coronary event. Having a past cardiovascular event raised the risk almost threefold (hazard ratio, 2.87) and carotid intima-media thickness also raised the risk significantly (HR, 1.61). After substituting carotid plaque for intima-media thickness, the investigators found a 2.5-fold increase in risk for unilateral plaque and almost a 6-fold increase in risk for bilateral plaque.

The findings confirmed that traditional demographic and cardiovascular risk factors also predict coronary events as would be expected. These include male gender (HR, 1.94), diabetes (HR, 2.24), and hypertension (HR, 1.56). Measures of RA severity, such as swollen joint counts (HR, 1.03) and cumulative prednisone dose of 20 g (HR, 2.12) also had predictive value.

Dr. Greenberg receives consulting fees from Genentech Inc.

NEW YORK – Imaging seems to be the sine qua non of determining cardiovascular disease risk in patients with rheumatoid arthritis.

Dr. Jeffrey D. Greenberg noted that, over the last 10–15 years, epidemiologic studies have shown patients with rheumatoid arthritis (RA) have a twofold increase in the risk of myocardial infarction and stroke and an increase in cardiovascular-related deaths.

“An important issue we face is how can we risk stratify our patients to predict who will develop cardiovascular disease? Imaging is a promising area that may help us develop biomarkers of risk or better understand pathophysiological mechanisms of RA,” said Dr. Greenberg, who is director of the Arthritis Translational Registry and Biorepository at NYU Hospital for Joint Diseases.

The need for precise tools with which to predict risk has become more urgent with the recently published findings that carotid ultrasound measurement of carotid intima-media thickness has been found to predict coronary events in patients with RA, independent of traditional cardiovascular risk factors and manifestations of RA.

The study, conducted by Dr. Matthew R. Evans and his associates at Brooke Army Medical Center, Fort Sam Houston, Tex., found that there appears to be a dose-dependent relationship between plaque and risk, with a 2.5-fold increase with unilateral plaque and 4.3-fold increase with bilateral carotid plaque, suggesting that atherosclerosis plays a significant role in acute coronary events in patients with RA (Arthritis Rheum. 2011 [doi:10.1002/art.30265]).

In discussing Dr. Evans's research at his presentation at the meeting, Dr. Greenberg said that this is the first study to demonstrate the predictive value of measuring carotid intima-media thickness and plaque for cardiovascular events in RA patients.

In the Evans study, carotid ultrasounds were performed on 636 RA patients as part of the prospective ORALE (Outcome of Rheumatoid Arthritis Longitudinal Evaluation). These patients were followed for 3,402 person-years and in that time 84 patients experienced 121 new or recurrent acute coronary syndrome (ACS) events, such as myocardial infarction, unstable angina, cardiac arrest, or death from ischemic heart disease. The rate of ACS events was 3.5/100 patient-years for this group. If only those without a prior history of ACS were analyzed, this group had 66 ACS events, with an incidence of 2.1 ACS/100 person-years.

Multivariate analysis of baseline factors associated with incident or recurrent acute coronary syndromes revealed that two markers were independent predictors of a subsequent coronary event. Having a past cardiovascular event raised the risk almost threefold (hazard ratio, 2.87) and carotid intima-media thickness also raised the risk significantly (HR, 1.61). After substituting carotid plaque for intima-media thickness, the investigators found a 2.5-fold increase in risk for unilateral plaque and almost a 6-fold increase in risk for bilateral plaque.

The findings confirmed that traditional demographic and cardiovascular risk factors also predict coronary events as would be expected. These include male gender (HR, 1.94), diabetes (HR, 2.24), and hypertension (HR, 1.56). Measures of RA severity, such as swollen joint counts (HR, 1.03) and cumulative prednisone dose of 20 g (HR, 2.12) also had predictive value.

Dr. Greenberg receives consulting fees from Genentech Inc.

NEW YORK – Because juvenile dermatomyositis is a rare disease and its symptoms often differ from those seen in adult dermatomyositis, pediatricians and family physicians may not recognize it and diagnosis can be delayed, Dr. Brian Feldman said.

He described a recent practice survey that found variability in juvenile dermatomyositis (JDM) treatment and, in the absence of randomized controlled trials, urged clinicians to consult newly created consensus protocols developed by rheumatologists and to gather data that may help to optimize treatment and minimize side effects in the future for those with JDM.

Dr. Feldman described a 15-year-old patient who had progressively deteriorated over a 3-year period despite being seen by pediatricians and dermatologists. His initial symptoms included fatigue and elevated muscle enzymes, including aspartate transaminase (AST), alanine transaminase (ALT), lactate dehydrogenase (LDH), and creatine phosphokinase (CPK).

Within a year, the patient developed a purple skin rash on his hands, elbows, and knees. He later developed Raynaud's syndrome and muscle weakness with decreased range of motion that interfered with participation in sports and other activities of daily living. Upon examination, he was quite thin with a scaly rash over his knuckles (Gottron's papules), healing skin ulcerations on his hands (from severe Raynaud's), periungual erythema, and lipodystrophy and erythema of his forearms. Magnetic resonance imaging showed acute inflammation of his shoulder girdle, said Dr. Feldman, chief of pediatric rheumatology at the Hospital for Sick Children in Toronto.

Dr. Feldman said that one of the best diagnostic clues for JDM comes from microscopic examination of nail folds. In this case, the patient had tortuous, bushy nail folds, dilated or missing capillaries (capillary density of 3 per mm of nailfold length while normal ranges from 7–11 per mm), and cuticular overgrowth indicative of JDM.

For rheumatologists, the different presentation seen with children compared with adults may hinder a correct diagnosis. For instance, calcinosis is seen much more often in children than adults. Children are less likely to have some of the systemic symptoms, such as fever, poor weight gain, and pulmonary effects, and they almost never have cardiac problems. Unlike adults with dermatomyositis or polymyositis who appear to have a fourfold increased risk of malignancy, very few cases of malignancy have been associated with JDM. Children are more likely to experience dysphonia/dysphagia but are as likely to have arthritic symptoms (around 58% of each group). Myositis-specific antibodies do not seem to play as important a diagnostic role for children as adults.

Children who develop JDM appear to have a better prognosis than adults. Although adults with myositis have appreciable mortality (about 10%) and progressive disability, findings from Dr. Feldman's group show that outcomes were often excellent in children. “We have not had a single death from JDM in 30 years,” he said.

About two-thirds of children with JDM have a polycyclic, chronic, unremitting disease course, but about one-third follow a monocyclic course and have disease symptoms that last for 2–3 years and then go away, with, or potentially even without, treatment. In a very small proportion of these patients, symptoms may return many years later. It is possible to predict which patients will follow a chronic course and which patients will have early remission. In his experience, the risk of persistent disease falls by half if Gottron's rash disappears within 3 months of diagnosis (odds ratio, 0.5; P = .0013) (Arthritis Rheum. 2008;58:3585–92).

The results of a recent practice survey by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) of almost 200 pediatric rheumatologists in North America found both similarities and differences in treatment approaches to JDM (J. Rheumatol. 2010;37:1953–61).

Corticosteroids and methotrexate (MTX) are mainstays of care, but the route and pattern of corticosteroid administration varied: 82% of respondents initially pulsed with high-dose intravenous methylprednisolone (IVMP) while 18% prescribed oral prednisone. MTX was used concurrently with steroids 84% of the time. Sixteen percent reported also using IV immunoglobulin, with or without MTX, for more severe disease, refractory disease, and prominent cutaneous disease. Hydroxychloroquine was given for milder cases, especially for rash, while cyclophosphamide was prescribed for ulcerative disease or for patients with pulmonary symptoms.

While these findings reflect the current prescribing practices of rheumatologists, there are almost no randomized controlled trials of medications for JDM, Dr. Feldman pointed out.

This reflects in part the rare nature of the disease, its complexity, the difficulty of conducting studies in children, and the high cost of randomized controlled trials. There have been studies using advanced analytic techniques to provide strong comparative data in lieu of an randomized controlled trial. One such study by Dr. Feldman and associates (Arthritis Rheum. 2008;59:989–95) showed that more aggressive corticosteroid therapy does not give better 3-year outcomes.

A recent randomized controlled trial presented at the annual meeting of the American College of Rheumatology looked at dermatomyositis in 76 adults and 48 children and found that 80% had a response to rituximab within a year of treatment. Interpretation of the study may have been limited by the design, which allowed patients to receive add-on medications during the trial.

In light of the CARRA study's findings of heterogeneity in the treatment of JDM and the absence of randomized controlled trials, a group of 12 pediatric rheumatologists met to study treatments in JDM using a new approach: by developing consensus treatment protocols (Arthritis Care Res. 2010;62:219–25).

“This is similar to what has been done in pediatric oncology,” said Dr. Feldman, who was one of the participants. “We are hoping physicians throughout the world will take these protocols off the shelf, and by using standardized doses, follow-up, and measurements, we will be able to accumulate enough evidence over time to know which is the best therapy.”

In brief, the group recommended three protocols for the treatment of patients with moderately severe JDM: pulse IVMP plus MTX; IVMP, MTX, plus IVIG; or oral prednisone plus MTX. The third protocol is the one followed most often at the Hospital for Sick Children. The treatment protocols are not intended as treatment recommendations, although it is hoped that a physician will choose to follow the standardized protocol that most closely reflects his or her preferred practice. It is presented as a “first step to allow comparison of different approaches to the treatment of JDM,” he said.

Dr. Feldman has done contracted research with Bayer Healthcare Pharmaceuticals. He has referenced unlabeled/unapproved uses of drugs or products in his presentation.

NEW YORK – Because juvenile dermatomyositis is a rare disease and its symptoms often differ from those seen in adult dermatomyositis, pediatricians and family physicians may not recognize it and diagnosis can be delayed, Dr. Brian Feldman said.

He described a recent practice survey that found variability in juvenile dermatomyositis (JDM) treatment and, in the absence of randomized controlled trials, urged clinicians to consult newly created consensus protocols developed by rheumatologists and to gather data that may help to optimize treatment and minimize side effects in the future for those with JDM.

Dr. Feldman described a 15-year-old patient who had progressively deteriorated over a 3-year period despite being seen by pediatricians and dermatologists. His initial symptoms included fatigue and elevated muscle enzymes, including aspartate transaminase (AST), alanine transaminase (ALT), lactate dehydrogenase (LDH), and creatine phosphokinase (CPK).

Within a year, the patient developed a purple skin rash on his hands, elbows, and knees. He later developed Raynaud's syndrome and muscle weakness with decreased range of motion that interfered with participation in sports and other activities of daily living. Upon examination, he was quite thin with a scaly rash over his knuckles (Gottron's papules), healing skin ulcerations on his hands (from severe Raynaud's), periungual erythema, and lipodystrophy and erythema of his forearms. Magnetic resonance imaging showed acute inflammation of his shoulder girdle, said Dr. Feldman, chief of pediatric rheumatology at the Hospital for Sick Children in Toronto.

Dr. Feldman said that one of the best diagnostic clues for JDM comes from microscopic examination of nail folds. In this case, the patient had tortuous, bushy nail folds, dilated or missing capillaries (capillary density of 3 per mm of nailfold length while normal ranges from 7–11 per mm), and cuticular overgrowth indicative of JDM.

For rheumatologists, the different presentation seen with children compared with adults may hinder a correct diagnosis. For instance, calcinosis is seen much more often in children than adults. Children are less likely to have some of the systemic symptoms, such as fever, poor weight gain, and pulmonary effects, and they almost never have cardiac problems. Unlike adults with dermatomyositis or polymyositis who appear to have a fourfold increased risk of malignancy, very few cases of malignancy have been associated with JDM. Children are more likely to experience dysphonia/dysphagia but are as likely to have arthritic symptoms (around 58% of each group). Myositis-specific antibodies do not seem to play as important a diagnostic role for children as adults.

Children who develop JDM appear to have a better prognosis than adults. Although adults with myositis have appreciable mortality (about 10%) and progressive disability, findings from Dr. Feldman's group show that outcomes were often excellent in children. “We have not had a single death from JDM in 30 years,” he said.

About two-thirds of children with JDM have a polycyclic, chronic, unremitting disease course, but about one-third follow a monocyclic course and have disease symptoms that last for 2–3 years and then go away, with, or potentially even without, treatment. In a very small proportion of these patients, symptoms may return many years later. It is possible to predict which patients will follow a chronic course and which patients will have early remission. In his experience, the risk of persistent disease falls by half if Gottron's rash disappears within 3 months of diagnosis (odds ratio, 0.5; P = .0013) (Arthritis Rheum. 2008;58:3585–92).

The results of a recent practice survey by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) of almost 200 pediatric rheumatologists in North America found both similarities and differences in treatment approaches to JDM (J. Rheumatol. 2010;37:1953–61).

Corticosteroids and methotrexate (MTX) are mainstays of care, but the route and pattern of corticosteroid administration varied: 82% of respondents initially pulsed with high-dose intravenous methylprednisolone (IVMP) while 18% prescribed oral prednisone. MTX was used concurrently with steroids 84% of the time. Sixteen percent reported also using IV immunoglobulin, with or without MTX, for more severe disease, refractory disease, and prominent cutaneous disease. Hydroxychloroquine was given for milder cases, especially for rash, while cyclophosphamide was prescribed for ulcerative disease or for patients with pulmonary symptoms.

While these findings reflect the current prescribing practices of rheumatologists, there are almost no randomized controlled trials of medications for JDM, Dr. Feldman pointed out.

This reflects in part the rare nature of the disease, its complexity, the difficulty of conducting studies in children, and the high cost of randomized controlled trials. There have been studies using advanced analytic techniques to provide strong comparative data in lieu of an randomized controlled trial. One such study by Dr. Feldman and associates (Arthritis Rheum. 2008;59:989–95) showed that more aggressive corticosteroid therapy does not give better 3-year outcomes.

A recent randomized controlled trial presented at the annual meeting of the American College of Rheumatology looked at dermatomyositis in 76 adults and 48 children and found that 80% had a response to rituximab within a year of treatment. Interpretation of the study may have been limited by the design, which allowed patients to receive add-on medications during the trial.

In light of the CARRA study's findings of heterogeneity in the treatment of JDM and the absence of randomized controlled trials, a group of 12 pediatric rheumatologists met to study treatments in JDM using a new approach: by developing consensus treatment protocols (Arthritis Care Res. 2010;62:219–25).

“This is similar to what has been done in pediatric oncology,” said Dr. Feldman, who was one of the participants. “We are hoping physicians throughout the world will take these protocols off the shelf, and by using standardized doses, follow-up, and measurements, we will be able to accumulate enough evidence over time to know which is the best therapy.”

In brief, the group recommended three protocols for the treatment of patients with moderately severe JDM: pulse IVMP plus MTX; IVMP, MTX, plus IVIG; or oral prednisone plus MTX. The third protocol is the one followed most often at the Hospital for Sick Children. The treatment protocols are not intended as treatment recommendations, although it is hoped that a physician will choose to follow the standardized protocol that most closely reflects his or her preferred practice. It is presented as a “first step to allow comparison of different approaches to the treatment of JDM,” he said.

Dr. Feldman has done contracted research with Bayer Healthcare Pharmaceuticals. He has referenced unlabeled/unapproved uses of drugs or products in his presentation.

NEW YORK – Because juvenile dermatomyositis is a rare disease and its symptoms often differ from those seen in adult dermatomyositis, pediatricians and family physicians may not recognize it and diagnosis can be delayed, Dr. Brian Feldman said.

He described a recent practice survey that found variability in juvenile dermatomyositis (JDM) treatment and, in the absence of randomized controlled trials, urged clinicians to consult newly created consensus protocols developed by rheumatologists and to gather data that may help to optimize treatment and minimize side effects in the future for those with JDM.

Dr. Feldman described a 15-year-old patient who had progressively deteriorated over a 3-year period despite being seen by pediatricians and dermatologists. His initial symptoms included fatigue and elevated muscle enzymes, including aspartate transaminase (AST), alanine transaminase (ALT), lactate dehydrogenase (LDH), and creatine phosphokinase (CPK).

Within a year, the patient developed a purple skin rash on his hands, elbows, and knees. He later developed Raynaud's syndrome and muscle weakness with decreased range of motion that interfered with participation in sports and other activities of daily living. Upon examination, he was quite thin with a scaly rash over his knuckles (Gottron's papules), healing skin ulcerations on his hands (from severe Raynaud's), periungual erythema, and lipodystrophy and erythema of his forearms. Magnetic resonance imaging showed acute inflammation of his shoulder girdle, said Dr. Feldman, chief of pediatric rheumatology at the Hospital for Sick Children in Toronto.

Dr. Feldman said that one of the best diagnostic clues for JDM comes from microscopic examination of nail folds. In this case, the patient had tortuous, bushy nail folds, dilated or missing capillaries (capillary density of 3 per mm of nailfold length while normal ranges from 7–11 per mm), and cuticular overgrowth indicative of JDM.

For rheumatologists, the different presentation seen with children compared with adults may hinder a correct diagnosis. For instance, calcinosis is seen much more often in children than adults. Children are less likely to have some of the systemic symptoms, such as fever, poor weight gain, and pulmonary effects, and they almost never have cardiac problems. Unlike adults with dermatomyositis or polymyositis who appear to have a fourfold increased risk of malignancy, very few cases of malignancy have been associated with JDM. Children are more likely to experience dysphonia/dysphagia but are as likely to have arthritic symptoms (around 58% of each group). Myositis-specific antibodies do not seem to play as important a diagnostic role for children as adults.

Children who develop JDM appear to have a better prognosis than adults. Although adults with myositis have appreciable mortality (about 10%) and progressive disability, findings from Dr. Feldman's group show that outcomes were often excellent in children. “We have not had a single death from JDM in 30 years,” he said.

About two-thirds of children with JDM have a polycyclic, chronic, unremitting disease course, but about one-third follow a monocyclic course and have disease symptoms that last for 2–3 years and then go away, with, or potentially even without, treatment. In a very small proportion of these patients, symptoms may return many years later. It is possible to predict which patients will follow a chronic course and which patients will have early remission. In his experience, the risk of persistent disease falls by half if Gottron's rash disappears within 3 months of diagnosis (odds ratio, 0.5; P = .0013) (Arthritis Rheum. 2008;58:3585–92).

The results of a recent practice survey by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) of almost 200 pediatric rheumatologists in North America found both similarities and differences in treatment approaches to JDM (J. Rheumatol. 2010;37:1953–61).

Corticosteroids and methotrexate (MTX) are mainstays of care, but the route and pattern of corticosteroid administration varied: 82% of respondents initially pulsed with high-dose intravenous methylprednisolone (IVMP) while 18% prescribed oral prednisone. MTX was used concurrently with steroids 84% of the time. Sixteen percent reported also using IV immunoglobulin, with or without MTX, for more severe disease, refractory disease, and prominent cutaneous disease. Hydroxychloroquine was given for milder cases, especially for rash, while cyclophosphamide was prescribed for ulcerative disease or for patients with pulmonary symptoms.

While these findings reflect the current prescribing practices of rheumatologists, there are almost no randomized controlled trials of medications for JDM, Dr. Feldman pointed out.

This reflects in part the rare nature of the disease, its complexity, the difficulty of conducting studies in children, and the high cost of randomized controlled trials. There have been studies using advanced analytic techniques to provide strong comparative data in lieu of an randomized controlled trial. One such study by Dr. Feldman and associates (Arthritis Rheum. 2008;59:989–95) showed that more aggressive corticosteroid therapy does not give better 3-year outcomes.

A recent randomized controlled trial presented at the annual meeting of the American College of Rheumatology looked at dermatomyositis in 76 adults and 48 children and found that 80% had a response to rituximab within a year of treatment. Interpretation of the study may have been limited by the design, which allowed patients to receive add-on medications during the trial.

In light of the CARRA study's findings of heterogeneity in the treatment of JDM and the absence of randomized controlled trials, a group of 12 pediatric rheumatologists met to study treatments in JDM using a new approach: by developing consensus treatment protocols (Arthritis Care Res. 2010;62:219–25).

“This is similar to what has been done in pediatric oncology,” said Dr. Feldman, who was one of the participants. “We are hoping physicians throughout the world will take these protocols off the shelf, and by using standardized doses, follow-up, and measurements, we will be able to accumulate enough evidence over time to know which is the best therapy.”

In brief, the group recommended three protocols for the treatment of patients with moderately severe JDM: pulse IVMP plus MTX; IVMP, MTX, plus IVIG; or oral prednisone plus MTX. The third protocol is the one followed most often at the Hospital for Sick Children. The treatment protocols are not intended as treatment recommendations, although it is hoped that a physician will choose to follow the standardized protocol that most closely reflects his or her preferred practice. It is presented as a “first step to allow comparison of different approaches to the treatment of JDM,” he said.

Dr. Feldman has done contracted research with Bayer Healthcare Pharmaceuticals. He has referenced unlabeled/unapproved uses of drugs or products in his presentation.

NEW YORK – Because juvenile dermatomyositis is a rare disease and its symptoms often differ from those seen in adult dermatomyositis, pediatricians and family physicians may not recognize it and diagnosis can be delayed, Dr. Brian Feldman said.

He described a recent practice survey that found variability in juvenile dermatomyositis (JDM) treatment and, in the absence of randomized controlled trials, urged clinicians to consult newly created consensus protocols developed by rheumatologists and to gather data that may help to optimize treatment and minimize side effects in the future for those with JDM.

Dr. Feldman described a 15-year-old patient who had progressively deteriorated over a 3-year period despite being seen by pediatricians and dermatologists. His initial symptoms included fatigue and elevated muscle enzymes, including aspartate transaminase (AST), alanine transaminase (ALT), lactate dehydrogenase (LDH), and creatine phosphokinase (CPK).

Within a year, the patient developed a purple skin rash on his hands, elbows, and knees. He later developed Raynaud’s syndrome and muscle weakness with decreased range of motion that interfered with participation in sports and other activities of daily living. Upon examination, he was quite thin with a scaly rash over his knuckles (Gottron’s papules), healing skin ulcerations on his hands (from severe Raynaud’s), periungual erythema, and lipodystrophy and erythema of his forearms. Magnetic resonance imaging showed acute inflammation of his shoulder girdle, said Dr. Feldman, chief of pediatric rheumatology at the Hospital for Sick Children in Toronto.

Dr. Feldman said that one of the best diagnostic clues for JDM comes from microscopic examination of nail folds. In this case, the patient had tortuous, bushy nail folds, dilated or missing capillaries (capillary density of 3 per mm of nailfold length while normal ranges from 7-11 per mm), and cuticular overgrowth indicative of JDM.

For rheumatologists, the different presentation seen with children compared with adults may hinder a correct diagnosis. For instance, calcinosis is seen much more frequently in children than adults. Children are less likely to have some of the systemic symptoms, such as fever, poor weight gain, and pulmonary effects, and they almost never have cardiac problems. Unlike adults with dermatomyositis or polymyositis who appear to have a fourfold increased risk of malignancy, very few cases of malignancy have been associated with JDM. Children are more likely to experience dysphonia/dysphagia but are as likely to have arthritic symptoms (around 58% of each group). Myositis specific antibodies do not seem to play as important a diagnostic role for children as adults.

Children who develop JDM appear to have a better prognosis than adults. Although adults with myositis have appreciable mortality (about 10%) and progressive disability, which may be related to treatment of a long-term condition such as avascular necrosis or osteoporosis with compression fracture, findings from Dr. Feldman’s group show that outcomes were often excellent in children. "We have not had a single death from JDM in 30 years," he said.

About two-thirds of children with JDM have a polycyclic, chronic, unremitting disease course, but about one-third follow a monocyclic course and have disease symptoms that last for 2-3 years and then go away, with, or potentially even without, treatment. In a very small proportion of these patients, symptoms may return many years later. "The message here is that when the disease goes away, it almost certainly is gone forever," Dr. Feldman noted.

It is possible to predict which patients will follow a chronic course and which patients will have early remission. In his experience, the risk of persistent disease falls by half if Gottron’s rash disappears within 3 months of diagnosis (odds ratio, 0.5; P = .0013) (Arthritis Rheum. 2008;58:3585-92).

The results of a recent practice survey by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) of almost 200 pediatric rheumatologists in North America found both similarities and differences in treatment approaches to JDM (J. Rheumatol. 2010;37:1953-61).

Corticosteroids and methotrexate (MTX) are mainstays of care, but the route and pattern of corticosteroid administration varied: 82% of respondents initially pulsed with high-dose intravenous methylprednisolone (IVMP) while 18% prescribed oral prednisone. MTX was used concurrently with steroids 84% of the time. Sixteen percent reported also using IV immunoglobulin, with or without MTX, for more severe disease, refractory disease, and prominent cutaneous disease. Hydroxychloroquine was given for milder cases, especially for rash, while cyclophosphamide was prescribed for ulcerative disease or for patients with pulmonary symptoms.

While these findings reflect the current prescribing practices of rheumatologists, there are almost no randomized controlled trials of medications for JDM, Dr. Feldman pointed out.

This reflects in part the rare nature of the disease, its complexity, the difficulty of conducting studies in children, and the high cost of randomized controlled trials. There have been studies using advanced analytic techniques to provide strong comparative data in lieu of an randomized controlled trial. One such study by Dr. Feldman and associates (Arthritis Rheum. 2008;59:989-95) showed that more aggressive corticosteroid therapy does not give better 3-year outcomes.

A recent randomized controlled trial presented at the annual meeting of the American College of Rheumatology looked at dermatomyositis in 76 adults and 48 children and found that 80% had a response to rituximab within a year of treatment. Interpretation of the study may have been limited by the design, which allowed patients to receive add-on medications during the trial.

In light of the CARRA study’s findings of heterogeneity in the treatment of JDM and the absence of randomized controlled trials, a group of 12 pediatric rheumatologists met to study treatments in JDM using a new approach: by developing consensus treatment protocols (Arthritis Care Res. 2010;62:219-25).

"This is similar to what has been done in pediatric oncology," said Dr. Feldman, who was one of the participants. "We are hoping physicians throughout the world will take these protocols off the shelf, and by using standardized doses, follow-up, and measurements, we will be able to accumulate enough evidence over time to know which is the best therapy."

In brief, the group recommended three protocols for the treatment of patients with moderately severe JDM: pulse IVMP plus MTX; IVMP, MTX, plus IVIG; or oral prednisone plus MTX. The third protocol is the one followed most often at the Hospital for Sick Children. The treatment protocols are not intended as treatment recommendations, although it is hoped that a physician will choose to follow the standardized protocol that most closely reflects his or her preferred practice. It is presented as a "first step to allow comparison of different approaches to the treatment of JDM," he said.

Dr. Feldman has done contracted research with Bayer Healthcare Pharmaceuticals. He referenced unlabeled/unapproved uses of drugs or products in his presentation.

NEW YORK – Because juvenile dermatomyositis is a rare disease and its symptoms often differ from those seen in adult dermatomyositis, pediatricians and family physicians may not recognize it and diagnosis can be delayed, Dr. Brian Feldman said.

He described a recent practice survey that found variability in juvenile dermatomyositis (JDM) treatment and, in the absence of randomized controlled trials, urged clinicians to consult newly created consensus protocols developed by rheumatologists and to gather data that may help to optimize treatment and minimize side effects in the future for those with JDM.

Dr. Feldman described a 15-year-old patient who had progressively deteriorated over a 3-year period despite being seen by pediatricians and dermatologists. His initial symptoms included fatigue and elevated muscle enzymes, including aspartate transaminase (AST), alanine transaminase (ALT), lactate dehydrogenase (LDH), and creatine phosphokinase (CPK).

Within a year, the patient developed a purple skin rash on his hands, elbows, and knees. He later developed Raynaud’s syndrome and muscle weakness with decreased range of motion that interfered with participation in sports and other activities of daily living. Upon examination, he was quite thin with a scaly rash over his knuckles (Gottron’s papules), healing skin ulcerations on his hands (from severe Raynaud’s), periungual erythema, and lipodystrophy and erythema of his forearms. Magnetic resonance imaging showed acute inflammation of his shoulder girdle, said Dr. Feldman, chief of pediatric rheumatology at the Hospital for Sick Children in Toronto.

Dr. Feldman said that one of the best diagnostic clues for JDM comes from microscopic examination of nail folds. In this case, the patient had tortuous, bushy nail folds, dilated or missing capillaries (capillary density of 3 per mm of nailfold length while normal ranges from 7-11 per mm), and cuticular overgrowth indicative of JDM.

For rheumatologists, the different presentation seen with children compared with adults may hinder a correct diagnosis. For instance, calcinosis is seen much more frequently in children than adults. Children are less likely to have some of the systemic symptoms, such as fever, poor weight gain, and pulmonary effects, and they almost never have cardiac problems. Unlike adults with dermatomyositis or polymyositis who appear to have a fourfold increased risk of malignancy, very few cases of malignancy have been associated with JDM. Children are more likely to experience dysphonia/dysphagia but are as likely to have arthritic symptoms (around 58% of each group). Myositis specific antibodies do not seem to play as important a diagnostic role for children as adults.

Children who develop JDM appear to have a better prognosis than adults. Although adults with myositis have appreciable mortality (about 10%) and progressive disability, which may be related to treatment of a long-term condition such as avascular necrosis or osteoporosis with compression fracture, findings from Dr. Feldman’s group show that outcomes were often excellent in children. "We have not had a single death from JDM in 30 years," he said.

About two-thirds of children with JDM have a polycyclic, chronic, unremitting disease course, but about one-third follow a monocyclic course and have disease symptoms that last for 2-3 years and then go away, with, or potentially even without, treatment. In a very small proportion of these patients, symptoms may return many years later. "The message here is that when the disease goes away, it almost certainly is gone forever," Dr. Feldman noted.

It is possible to predict which patients will follow a chronic course and which patients will have early remission. In his experience, the risk of persistent disease falls by half if Gottron’s rash disappears within 3 months of diagnosis (odds ratio, 0.5; P = .0013) (Arthritis Rheum. 2008;58:3585-92).

The results of a recent practice survey by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) of almost 200 pediatric rheumatologists in North America found both similarities and differences in treatment approaches to JDM (J. Rheumatol. 2010;37:1953-61).

Corticosteroids and methotrexate (MTX) are mainstays of care, but the route and pattern of corticosteroid administration varied: 82% of respondents initially pulsed with high-dose intravenous methylprednisolone (IVMP) while 18% prescribed oral prednisone. MTX was used concurrently with steroids 84% of the time. Sixteen percent reported also using IV immunoglobulin, with or without MTX, for more severe disease, refractory disease, and prominent cutaneous disease. Hydroxychloroquine was given for milder cases, especially for rash, while cyclophosphamide was prescribed for ulcerative disease or for patients with pulmonary symptoms.

While these findings reflect the current prescribing practices of rheumatologists, there are almost no randomized controlled trials of medications for JDM, Dr. Feldman pointed out.

This reflects in part the rare nature of the disease, its complexity, the difficulty of conducting studies in children, and the high cost of randomized controlled trials. There have been studies using advanced analytic techniques to provide strong comparative data in lieu of an randomized controlled trial. One such study by Dr. Feldman and associates (Arthritis Rheum. 2008;59:989-95) showed that more aggressive corticosteroid therapy does not give better 3-year outcomes.

A recent randomized controlled trial presented at the annual meeting of the American College of Rheumatology looked at dermatomyositis in 76 adults and 48 children and found that 80% had a response to rituximab within a year of treatment. Interpretation of the study may have been limited by the design, which allowed patients to receive add-on medications during the trial.

In light of the CARRA study’s findings of heterogeneity in the treatment of JDM and the absence of randomized controlled trials, a group of 12 pediatric rheumatologists met to study treatments in JDM using a new approach: by developing consensus treatment protocols (Arthritis Care Res. 2010;62:219-25).

"This is similar to what has been done in pediatric oncology," said Dr. Feldman, who was one of the participants. "We are hoping physicians throughout the world will take these protocols off the shelf, and by using standardized doses, follow-up, and measurements, we will be able to accumulate enough evidence over time to know which is the best therapy."

In brief, the group recommended three protocols for the treatment of patients with moderately severe JDM: pulse IVMP plus MTX; IVMP, MTX, plus IVIG; or oral prednisone plus MTX. The third protocol is the one followed most often at the Hospital for Sick Children. The treatment protocols are not intended as treatment recommendations, although it is hoped that a physician will choose to follow the standardized protocol that most closely reflects his or her preferred practice. It is presented as a "first step to allow comparison of different approaches to the treatment of JDM," he said.

Dr. Feldman has done contracted research with Bayer Healthcare Pharmaceuticals. He referenced unlabeled/unapproved uses of drugs or products in his presentation.

NEW YORK – Because juvenile dermatomyositis is a rare disease and its symptoms often differ from those seen in adult dermatomyositis, pediatricians and family physicians may not recognize it and diagnosis can be delayed, Dr. Brian Feldman said.

He described a recent practice survey that found variability in juvenile dermatomyositis (JDM) treatment and, in the absence of randomized controlled trials, urged clinicians to consult newly created consensus protocols developed by rheumatologists and to gather data that may help to optimize treatment and minimize side effects in the future for those with JDM.

Dr. Feldman described a 15-year-old patient who had progressively deteriorated over a 3-year period despite being seen by pediatricians and dermatologists. His initial symptoms included fatigue and elevated muscle enzymes, including aspartate transaminase (AST), alanine transaminase (ALT), lactate dehydrogenase (LDH), and creatine phosphokinase (CPK).

Within a year, the patient developed a purple skin rash on his hands, elbows, and knees. He later developed Raynaud’s syndrome and muscle weakness with decreased range of motion that interfered with participation in sports and other activities of daily living. Upon examination, he was quite thin with a scaly rash over his knuckles (Gottron’s papules), healing skin ulcerations on his hands (from severe Raynaud’s), periungual erythema, and lipodystrophy and erythema of his forearms. Magnetic resonance imaging showed acute inflammation of his shoulder girdle, said Dr. Feldman, chief of pediatric rheumatology at the Hospital for Sick Children in Toronto.

Dr. Feldman said that one of the best diagnostic clues for JDM comes from microscopic examination of nail folds. In this case, the patient had tortuous, bushy nail folds, dilated or missing capillaries (capillary density of 3 per mm of nailfold length while normal ranges from 7-11 per mm), and cuticular overgrowth indicative of JDM.

For rheumatologists, the different presentation seen with children compared with adults may hinder a correct diagnosis. For instance, calcinosis is seen much more frequently in children than adults. Children are less likely to have some of the systemic symptoms, such as fever, poor weight gain, and pulmonary effects, and they almost never have cardiac problems. Unlike adults with dermatomyositis or polymyositis who appear to have a fourfold increased risk of malignancy, very few cases of malignancy have been associated with JDM. Children are more likely to experience dysphonia/dysphagia but are as likely to have arthritic symptoms (around 58% of each group). Myositis specific antibodies do not seem to play as important a diagnostic role for children as adults.

Children who develop JDM appear to have a better prognosis than adults. Although adults with myositis have appreciable mortality (about 10%) and progressive disability, which may be related to treatment of a long-term condition such as avascular necrosis or osteoporosis with compression fracture, findings from Dr. Feldman’s group show that outcomes were often excellent in children. "We have not had a single death from JDM in 30 years," he said.

About two-thirds of children with JDM have a polycyclic, chronic, unremitting disease course, but about one-third follow a monocyclic course and have disease symptoms that last for 2-3 years and then go away, with, or potentially even without, treatment. In a very small proportion of these patients, symptoms may return many years later. "The message here is that when the disease goes away, it almost certainly is gone forever," Dr. Feldman noted.

It is possible to predict which patients will follow a chronic course and which patients will have early remission. In his experience, the risk of persistent disease falls by half if Gottron’s rash disappears within 3 months of diagnosis (odds ratio, 0.5; P = .0013) (Arthritis Rheum. 2008;58:3585-92).

The results of a recent practice survey by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) of almost 200 pediatric rheumatologists in North America found both similarities and differences in treatment approaches to JDM (J. Rheumatol. 2010;37:1953-61).

Corticosteroids and methotrexate (MTX) are mainstays of care, but the route and pattern of corticosteroid administration varied: 82% of respondents initially pulsed with high-dose intravenous methylprednisolone (IVMP) while 18% prescribed oral prednisone. MTX was used concurrently with steroids 84% of the time. Sixteen percent reported also using IV immunoglobulin, with or without MTX, for more severe disease, refractory disease, and prominent cutaneous disease. Hydroxychloroquine was given for milder cases, especially for rash, while cyclophosphamide was prescribed for ulcerative disease or for patients with pulmonary symptoms.

While these findings reflect the current prescribing practices of rheumatologists, there are almost no randomized controlled trials of medications for JDM, Dr. Feldman pointed out.

This reflects in part the rare nature of the disease, its complexity, the difficulty of conducting studies in children, and the high cost of randomized controlled trials. There have been studies using advanced analytic techniques to provide strong comparative data in lieu of an randomized controlled trial. One such study by Dr. Feldman and associates (Arthritis Rheum. 2008;59:989-95) showed that more aggressive corticosteroid therapy does not give better 3-year outcomes.

A recent randomized controlled trial presented at the annual meeting of the American College of Rheumatology looked at dermatomyositis in 76 adults and 48 children and found that 80% had a response to rituximab within a year of treatment. Interpretation of the study may have been limited by the design, which allowed patients to receive add-on medications during the trial.

In light of the CARRA study’s findings of heterogeneity in the treatment of JDM and the absence of randomized controlled trials, a group of 12 pediatric rheumatologists met to study treatments in JDM using a new approach: by developing consensus treatment protocols (Arthritis Care Res. 2010;62:219-25).

"This is similar to what has been done in pediatric oncology," said Dr. Feldman, who was one of the participants. "We are hoping physicians throughout the world will take these protocols off the shelf, and by using standardized doses, follow-up, and measurements, we will be able to accumulate enough evidence over time to know which is the best therapy."

In brief, the group recommended three protocols for the treatment of patients with moderately severe JDM: pulse IVMP plus MTX; IVMP, MTX, plus IVIG; or oral prednisone plus MTX. The third protocol is the one followed most often at the Hospital for Sick Children. The treatment protocols are not intended as treatment recommendations, although it is hoped that a physician will choose to follow the standardized protocol that most closely reflects his or her preferred practice. It is presented as a "first step to allow comparison of different approaches to the treatment of JDM," he said.

Dr. Feldman has done contracted research with Bayer Healthcare Pharmaceuticals. He referenced unlabeled/unapproved uses of drugs or products in his presentation.

NEW YORK – Because juvenile dermatomyositis is a rare disease and its symptoms often differ from those seen in adult dermatomyositis, pediatricians and family physicians may not recognize it and diagnosis can be delayed, Dr. Brian Feldman said.

He described a recent practice survey that found variability in juvenile dermatomyositis (JDM) treatment and, in the absence of randomized controlled trials, urged clinicians to consult newly created consensus protocols developed by rheumatologists and to gather data that may help to optimize treatment and minimize side effects in the future for those with JDM.

Dr. Feldman described a 15-year-old patient who had progressively deteriorated over a 3-year period despite being seen by pediatricians and dermatologists. His initial symptoms included fatigue and elevated muscle enzymes, including aspartate transaminase (AST), alanine transaminase (ALT), lactate dehydrogenase (LDH), and creatine phosphokinase (CPK).

Within a year, the patient developed a purple skin rash on his hands, elbows, and knees. He later developed Raynaud’s syndrome and muscle weakness with decreased range of motion that interfered with participation in sports and other activities of daily living. Upon examination, he was quite thin with a scaly rash over his knuckles (Gottron’s papules), healing skin ulcerations on his hands (from severe Raynaud’s), periungual erythema, and lipodystrophy and erythema of his forearms. Magnetic resonance imaging showed acute inflammation of his shoulder girdle, said Dr. Feldman, chief of pediatric rheumatology at the Hospital for Sick Children in Toronto.

Dr. Feldman said that one of the best diagnostic clues for JDM comes from microscopic examination of nail folds. In this case, the patient had tortuous, bushy nail folds, dilated or missing capillaries (capillary density of 3 per mm of nailfold length while normal ranges from 7-11 per mm), and cuticular overgrowth indicative of JDM.

For rheumatologists, the different presentation seen with children compared with adults may hinder a correct diagnosis. For instance, calcinosis is seen much more frequently in children than adults. Children are less likely to have some of the systemic symptoms, such as fever, poor weight gain, and pulmonary effects, and they almost never have cardiac problems. Unlike adults with dermatomyositis or polymyositis who appear to have a fourfold increased risk of malignancy, very few cases of malignancy have been associated with JDM. Children are more likely to experience dysphonia/dysphagia but are as likely to have arthritic symptoms (around 58% of each group). Myositis specific antibodies do not seem to play as important a diagnostic role for children as adults.

Children who develop JDM appear to have a better prognosis than adults. Although adults with myositis have appreciable mortality (about 10%) and progressive disability, which may be related to treatment of a long-term condition such as avascular necrosis or osteoporosis with compression fracture, findings from Dr. Feldman’s group show that outcomes were often excellent in children. "We have not had a single death from JDM in 30 years," he said.

About two-thirds of children with JDM have a polycyclic, chronic, unremitting disease course, but about one-third follow a monocyclic course and have disease symptoms that last for 2-3 years and then go away, with, or potentially even without, treatment. In a very small proportion of these patients, symptoms may return many years later. "The message here is that when the disease goes away, it almost certainly is gone forever," Dr. Feldman noted.

It is possible to predict which patients will follow a chronic course and which patients will have early remission. In his experience, the risk of persistent disease falls by half if Gottron’s rash disappears within 3 months of diagnosis (odds ratio, 0.5; P = .0013) (Arthritis Rheum. 2008;58:3585-92).

The results of a recent practice survey by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) of almost 200 pediatric rheumatologists in North America found both similarities and differences in treatment approaches to JDM (J. Rheumatol. 2010;37:1953-61).

Corticosteroids and methotrexate (MTX) are mainstays of care, but the route and pattern of corticosteroid administration varied: 82% of respondents initially pulsed with high-dose intravenous methylprednisolone (IVMP) while 18% prescribed oral prednisone. MTX was used concurrently with steroids 84% of the time. Sixteen percent reported also using IV immunoglobulin, with or without MTX, for more severe disease, refractory disease, and prominent cutaneous disease. Hydroxychloroquine was given for milder cases, especially for rash, while cyclophosphamide was prescribed for ulcerative disease or for patients with pulmonary symptoms.

While these findings reflect the current prescribing practices of rheumatologists, there are almost no randomized controlled trials of medications for JDM, Dr. Feldman pointed out.

This reflects in part the rare nature of the disease, its complexity, the difficulty of conducting studies in children, and the high cost of randomized controlled trials. There have been studies using advanced analytic techniques to provide strong comparative data in lieu of an randomized controlled trial. One such study by Dr. Feldman and associates (Arthritis Rheum. 2008;59:989-95) showed that more aggressive corticosteroid therapy does not give better 3-year outcomes.

A recent randomized controlled trial presented at the annual meeting of the American College of Rheumatology looked at dermatomyositis in 76 adults and 48 children and found that 80% had a response to rituximab within a year of treatment. Interpretation of the study may have been limited by the design, which allowed patients to receive add-on medications during the trial.

In light of the CARRA study’s findings of heterogeneity in the treatment of JDM and the absence of randomized controlled trials, a group of 12 pediatric rheumatologists met to study treatments in JDM using a new approach: by developing consensus treatment protocols (Arthritis Care Res. 2010;62:219-25).

"This is similar to what has been done in pediatric oncology," said Dr. Feldman, who was one of the participants. "We are hoping physicians throughout the world will take these protocols off the shelf, and by using standardized doses, follow-up, and measurements, we will be able to accumulate enough evidence over time to know which is the best therapy."

In brief, the group recommended three protocols for the treatment of patients with moderately severe JDM: pulse IVMP plus MTX; IVMP, MTX, plus IVIG; or oral prednisone plus MTX. The third protocol is the one followed most often at the Hospital for Sick Children. The treatment protocols are not intended as treatment recommendations, although it is hoped that a physician will choose to follow the standardized protocol that most closely reflects his or her preferred practice. It is presented as a "first step to allow comparison of different approaches to the treatment of JDM," he said.

Dr. Feldman has done contracted research with Bayer Healthcare Pharmaceuticals. He referenced unlabeled/unapproved uses of drugs or products in his presentation.

NEW YORK – Because juvenile dermatomyositis is a rare disease and its symptoms often differ from those seen in adult dermatomyositis, pediatricians and family physicians may not recognize it and diagnosis can be delayed, Dr. Brian Feldman said.

He described a recent practice survey that found variability in juvenile dermatomyositis (JDM) treatment and, in the absence of randomized controlled trials, urged clinicians to consult newly created consensus protocols developed by rheumatologists and to gather data that may help to optimize treatment and minimize side effects in the future for those with JDM.

Dr. Feldman described a 15-year-old patient who had progressively deteriorated over a 3-year period despite being seen by pediatricians and dermatologists. His initial symptoms included fatigue and elevated muscle enzymes, including aspartate transaminase (AST), alanine transaminase (ALT), lactate dehydrogenase (LDH), and creatine phosphokinase (CPK).

Within a year, the patient developed a purple skin rash on his hands, elbows, and knees. He later developed Raynaud’s syndrome and muscle weakness with decreased range of motion that interfered with participation in sports and other activities of daily living. Upon examination, he was quite thin with a scaly rash over his knuckles (Gottron’s papules), healing skin ulcerations on his hands (from severe Raynaud’s), periungual erythema, and lipodystrophy and erythema of his forearms. Magnetic resonance imaging showed acute inflammation of his shoulder girdle, said Dr. Feldman, chief of pediatric rheumatology at the Hospital for Sick Children in Toronto.

Dr. Feldman said that one of the best diagnostic clues for JDM comes from microscopic examination of nail folds. In this case, the patient had tortuous, bushy nail folds, dilated or missing capillaries (capillary density of 3 per mm of nailfold length while normal ranges from 7-11 per mm), and cuticular overgrowth indicative of JDM.

For rheumatologists, the different presentation seen with children compared with adults may hinder a correct diagnosis. For instance, calcinosis is seen much more frequently in children than adults. Children are less likely to have some of the systemic symptoms, such as fever, poor weight gain, and pulmonary effects, and they almost never have cardiac problems. Unlike adults with dermatomyositis or polymyositis who appear to have a fourfold increased risk of malignancy, very few cases of malignancy have been associated with JDM. Children are more likely to experience dysphonia/dysphagia but are as likely to have arthritic symptoms (around 58% of each group). Myositis specific antibodies do not seem to play as important a diagnostic role for children as adults.

Children who develop JDM appear to have a better prognosis than adults. Although adults with myositis have appreciable mortality (about 10%) and progressive disability, which may be related to treatment of a long-term condition such as avascular necrosis or osteoporosis with compression fracture, findings from Dr. Feldman’s group show that outcomes were often excellent in children. "We have not had a single death from JDM in 30 years," he said.

About two-thirds of children with JDM have a polycyclic, chronic, unremitting disease course, but about one-third follow a monocyclic course and have disease symptoms that last for 2-3 years and then go away, with, or potentially even without, treatment. In a very small proportion of these patients, symptoms may return many years later. "The message here is that when the disease goes away, it almost certainly is gone forever," Dr. Feldman noted.

It is possible to predict which patients will follow a chronic course and which patients will have early remission. In his experience, the risk of persistent disease falls by half if Gottron’s rash disappears within 3 months of diagnosis (odds ratio, 0.5; P = .0013) (Arthritis Rheum. 2008;58:3585-92).

The results of a recent practice survey by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) of almost 200 pediatric rheumatologists in North America found both similarities and differences in treatment approaches to JDM (J. Rheumatol. 2010;37:1953-61).

Corticosteroids and methotrexate (MTX) are mainstays of care, but the route and pattern of corticosteroid administration varied: 82% of respondents initially pulsed with high-dose intravenous methylprednisolone (IVMP) while 18% prescribed oral prednisone. MTX was used concurrently with steroids 84% of the time. Sixteen percent reported also using IV immunoglobulin, with or without MTX, for more severe disease, refractory disease, and prominent cutaneous disease. Hydroxychloroquine was given for milder cases, especially for rash, while cyclophosphamide was prescribed for ulcerative disease or for patients with pulmonary symptoms.

While these findings reflect the current prescribing practices of rheumatologists, there are almost no randomized controlled trials of medications for JDM, Dr. Feldman pointed out.

This reflects in part the rare nature of the disease, its complexity, the difficulty of conducting studies in children, and the high cost of randomized controlled trials. There have been studies using advanced analytic techniques to provide strong comparative data in lieu of an randomized controlled trial. One such study by Dr. Feldman and associates (Arthritis Rheum. 2008;59:989-95) showed that more aggressive corticosteroid therapy does not give better 3-year outcomes.

A recent randomized controlled trial presented at the annual meeting of the American College of Rheumatology looked at dermatomyositis in 76 adults and 48 children and found that 80% had a response to rituximab within a year of treatment. Interpretation of the study may have been limited by the design, which allowed patients to receive add-on medications during the trial.

In light of the CARRA study’s findings of heterogeneity in the treatment of JDM and the absence of randomized controlled trials, a group of 12 pediatric rheumatologists met to study treatments in JDM using a new approach: by developing consensus treatment protocols (Arthritis Care Res. 2010;62:219-25).

"This is similar to what has been done in pediatric oncology," said Dr. Feldman, who was one of the participants. "We are hoping physicians throughout the world will take these protocols off the shelf, and by using standardized doses, follow-up, and measurements, we will be able to accumulate enough evidence over time to know which is the best therapy."

In brief, the group recommended three protocols for the treatment of patients with moderately severe JDM: pulse IVMP plus MTX; IVMP, MTX, plus IVIG; or oral prednisone plus MTX. The third protocol is the one followed most often at the Hospital for Sick Children. The treatment protocols are not intended as treatment recommendations, although it is hoped that a physician will choose to follow the standardized protocol that most closely reflects his or her preferred practice. It is presented as a "first step to allow comparison of different approaches to the treatment of JDM," he said.

Dr. Feldman has done contracted research with Bayer Healthcare Pharmaceuticals. He referenced unlabeled/unapproved uses of drugs or products in his presentation.

NEW YORK – Because juvenile dermatomyositis is a rare disease and its symptoms often differ from those seen in adult dermatomyositis, pediatricians and family physicians may not recognize it and diagnosis can be delayed, Dr. Brian Feldman said.

He described a recent practice survey that found variability in juvenile dermatomyositis (JDM) treatment and, in the absence of randomized controlled trials, urged clinicians to consult newly created consensus protocols developed by rheumatologists and to gather data that may help to optimize treatment and minimize side effects in the future for those with JDM.

Dr. Feldman described a 15-year-old patient who had progressively deteriorated over a 3-year period despite being seen by pediatricians and dermatologists. His initial symptoms included fatigue and elevated muscle enzymes, including aspartate transaminase (AST), alanine transaminase (ALT), lactate dehydrogenase (LDH), and creatine phosphokinase (CPK).

Within a year, the patient developed a purple skin rash on his hands, elbows, and knees. He later developed Raynaud’s syndrome and muscle weakness with decreased range of motion that interfered with participation in sports and other activities of daily living. Upon examination, he was quite thin with a scaly rash over his knuckles (Gottron’s papules), healing skin ulcerations on his hands (from severe Raynaud’s), periungual erythema, and lipodystrophy and erythema of his forearms. Magnetic resonance imaging showed acute inflammation of his shoulder girdle, said Dr. Feldman, chief of pediatric rheumatology at the Hospital for Sick Children in Toronto.

Dr. Feldman said that one of the best diagnostic clues for JDM comes from microscopic examination of nail folds. In this case, the patient had tortuous, bushy nail folds, dilated or missing capillaries (capillary density of 3 per mm of nailfold length while normal ranges from 7-11 per mm), and cuticular overgrowth indicative of JDM.

For rheumatologists, the different presentation seen with children compared with adults may hinder a correct diagnosis. For instance, calcinosis is seen much more frequently in children than adults. Children are less likely to have some of the systemic symptoms, such as fever, poor weight gain, and pulmonary effects, and they almost never have cardiac problems. Unlike adults with dermatomyositis or polymyositis who appear to have a fourfold increased risk of malignancy, very few cases of malignancy have been associated with JDM. Children are more likely to experience dysphonia/dysphagia but are as likely to have arthritic symptoms (around 58% of each group). Myositis specific antibodies do not seem to play as important a diagnostic role for children as adults.

Children who develop JDM appear to have a better prognosis than adults. Although adults with myositis have appreciable mortality (about 10%) and progressive disability, which may be related to treatment of a long-term condition such as avascular necrosis or osteoporosis with compression fracture, findings from Dr. Feldman’s group show that outcomes were often excellent in children. "We have not had a single death from JDM in 30 years," he said.

About two-thirds of children with JDM have a polycyclic, chronic, unremitting disease course, but about one-third follow a monocyclic course and have disease symptoms that last for 2-3 years and then go away, with, or potentially even without, treatment. In a very small proportion of these patients, symptoms may return many years later. "The message here is that when the disease goes away, it almost certainly is gone forever," Dr. Feldman noted.

It is possible to predict which patients will follow a chronic course and which patients will have early remission. In his experience, the risk of persistent disease falls by half if Gottron’s rash disappears within 3 months of diagnosis (odds ratio, 0.5; P = .0013) (Arthritis Rheum. 2008;58:3585-92).

The results of a recent practice survey by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) of almost 200 pediatric rheumatologists in North America found both similarities and differences in treatment approaches to JDM (J. Rheumatol. 2010;37:1953-61).

Corticosteroids and methotrexate (MTX) are mainstays of care, but the route and pattern of corticosteroid administration varied: 82% of respondents initially pulsed with high-dose intravenous methylprednisolone (IVMP) while 18% prescribed oral prednisone. MTX was used concurrently with steroids 84% of the time. Sixteen percent reported also using IV immunoglobulin, with or without MTX, for more severe disease, refractory disease, and prominent cutaneous disease. Hydroxychloroquine was given for milder cases, especially for rash, while cyclophosphamide was prescribed for ulcerative disease or for patients with pulmonary symptoms.

While these findings reflect the current prescribing practices of rheumatologists, there are almost no randomized controlled trials of medications for JDM, Dr. Feldman pointed out.

This reflects in part the rare nature of the disease, its complexity, the difficulty of conducting studies in children, and the high cost of randomized controlled trials. There have been studies using advanced analytic techniques to provide strong comparative data in lieu of an randomized controlled trial. One such study by Dr. Feldman and associates (Arthritis Rheum. 2008;59:989-95) showed that more aggressive corticosteroid therapy does not give better 3-year outcomes.

A recent randomized controlled trial presented at the annual meeting of the American College of Rheumatology looked at dermatomyositis in 76 adults and 48 children and found that 80% had a response to rituximab within a year of treatment. Interpretation of the study may have been limited by the design, which allowed patients to receive add-on medications during the trial.

In light of the CARRA study’s findings of heterogeneity in the treatment of JDM and the absence of randomized controlled trials, a group of 12 pediatric rheumatologists met to study treatments in JDM using a new approach: by developing consensus treatment protocols (Arthritis Care Res. 2010;62:219-25).

"This is similar to what has been done in pediatric oncology," said Dr. Feldman, who was one of the participants. "We are hoping physicians throughout the world will take these protocols off the shelf, and by using standardized doses, follow-up, and measurements, we will be able to accumulate enough evidence over time to know which is the best therapy."

In brief, the group recommended three protocols for the treatment of patients with moderately severe JDM: pulse IVMP plus MTX; IVMP, MTX, plus IVIG; or oral prednisone plus MTX. The third protocol is the one followed most often at the Hospital for Sick Children. The treatment protocols are not intended as treatment recommendations, although it is hoped that a physician will choose to follow the standardized protocol that most closely reflects his or her preferred practice. It is presented as a "first step to allow comparison of different approaches to the treatment of JDM," he said.

Dr. Feldman has done contracted research with Bayer Healthcare Pharmaceuticals. He referenced unlabeled/unapproved uses of drugs or products in his presentation.

NEW YORK – The good news in the management of childhood-onset systemic lupus erythematosus is that children who are diagnosed with the disease have a better prognosis and are living longer. The bad news is that over the course of a lifetime, affected children develop more morbidities and experience more medication-induced toxicities, which create additional management challenges, according to Dr. Phillip Kahn, who spoke at a meeting sponsored by New York University.

About 15%-20% of lupus cases present in childhood. Children younger than age 5 years rarely have the disease, but up to 40% of patients with childhood-onset SLE (cSLE) present prior to puberty, said Dr. Kahn of New York University. The incidence of cSLE is estimated to be 10-20 cases per 100,000 individuals, but the incidence is higher in blacks, at 200 cases per 100,000 individuals (Lupus 2007;16:546-9; 2005;14:83-8).

Often the diagnosis is delayed in children, as their clinical features do not fall within those defined by the 1997 American College of Rheumatology’s SLE criteria, which have not been validated in children. For example, Dr. Kahn described the case of a 13-year-old girl who presented with "constitutional symptoms" such as diffuse lymphadenopathy, malaise, weight loss, and fever – none of which are included in the 1997 criteria.

Mucocutaneous lesions are common in children, with up to 70% presenting with malar rash, 30% with alopecia, 66% with oral ulcers, and 10% with nasal ulcers. "Although nasal ulcers are less common in children than adults, they still may result in perforation of the septum," he said. Other common manifestations include painful polyarthritis, hepatosplenomegaly, headache, photosensitivity, and Raynaud’s phenomenon. Up to 40% of cSLE patients have neuropsychiatric symptoms, significantly more than do adults (Lupus 2008;17:314-22).

An important diagnostic tool for cSLE is a positive ANA (antinuclear antibody) finding, which is present in essentially all children with cSLE. In contrast, up to 10% of adults with SLE do not have a positive ANA, said Dr. Kahn.

Childhood-onset lupus may predict mortality in adults (Arthritis Care Res. [Hoboken] 2010;62:1152-9) and thus physicians must be vigilant about detecting, preventing, and aggressively treating some of its more dangerous manifestations. To prevent recurrent infection, Dr. Kahn recommends keeping a child up to date with immunizations, treating infections promptly when they occur, and considering PCP (pneumocystis carinii pneumonia) prophylaxis in the appropriate patient. Children with cSLE are also at increased risk for premature atherosclerosis, with dyslipidemia seen in 60%-85% of affected children (Nat. Clin. Pract. Rheumatol. 2008;4:258-65). Statin therapy may be appropriate in these children, especially to prevent long-term adverse events such as early MI. For example, adults – especially women – who had c SLE have increased incidence of MI when they reach their 30s (Arthritis Rheum. 2009;61:13-20). The ongoing APPLE (Atherosclerosis Prevention in Pediatric Lupus Erythematosus) trial is investigating, in greater detail, cardiovascular risk and the use of statins in cSLE (Lupus 2010;19:1315-25).

Because nephropathy is the main cause of mortality and morbidity in cSLE, it is important to monitor urinary markers of renal function. Nephropathy may take a greater toll on a pediatric patient, compared with adults; data show that children are more likely to have renal involvement, need hemodialysis and kidney transplantation, and require hospitalization and emergency department visits (Lupus 2008;17:314-22; Arthritis Rheum. 2009;61:13-20). To avoid end-stage renal disease, Dr. Kahn urges tight blood pressure control, as well as antihypertensive medication as necessary. "We know that 90% of the patients who ultimately develop renal disease will do so within the first 2 years of disease, so if you follow a patient for [more than 2] years and they do not develop the disease, the good news is they are much less likely to develop nephropathy," said Dr. Kahn.

In the 1950s, the 5-year survival rate for SLE in adults and children ranged from 18% to 70%; 30 years later, 5-year survival rose to 60%-93%, in large part because of the introduction of prednisone. Currently, prednisone is almost universally given to those with cSLE, and the proportion of children who are treated with prednisone is significantly higher than the proportion of adults with SLE (97% vs. 70%; P less than .0001) (Arthritis Rheum. 2008;58:556-62). The result is greater risk of osteoporosis, diabetes, and growth failure. In fact, up to 40% of cSLE patients have growth failure or short stature, in part because of chronic steroids.

That is why Dr. Kahn posed the question, "Can we move beyond steroids?" Until recently, the only Food and Drug Administration–approved medications for SLE were prednisone, hydroxychloroquine, and aspirin. Steroid-sparing immunomodulatory therapy is sometimes used off label when major organ (such as brain or kidney) involvement develops, but most evidence regarding the efficacy of these medications pertains to renal proliferative disease in adults.

Cyclophosphamide is often used for induction of nephritis, but 30% of patients may still develop end-stage renal disease despite treatment, said Dr. Kahn. Mycophenolate mofetil has been shown to have equal efficacy to cyclophosphamide in induction of remission of nephritis, but sufficient pediatric data are lacking. Azathioprine is effective for maintenance therapy of nephritis after the induction phase.

Other options that are sometimes used include intravenous immunoglobulin, methotrexate, calcineurin inhibitors (such as tacrolimus), plasmapheresis, and autologous stem cell transplantation/immunoablative therapy.

More recently, the focus has been on target-specific therapy for SLE with potentially fewer side effects. Rituximab, which targets B cells that have the CD20 surface antigen, is sometimes used to treat resistant patients, but there is no strong clinical evidence to demonstrate its effectiveness in cSLE (Arthritis Rheum. 2009;61:482-7; Clin. J. Am. Soc. Nephrol. 2009;4:579-87).

Abatacept, which targets T cells by binding to B7 peripheral membrane protein, has shown some clinical benefit in a phase IIB randomized clinical trial, and is undergoing clinical testing as an add-on induction therapy with cyclophosphamide (Arthritis Rheum. 2010;62:3077-87). Belimumab, a monoclonal antibody that targets BLyS (B-lymphocyte stimulator)/BAFF (B-cell activating factor), is the first FDA-approved medication for SLE in more than 50 years, based on recent clinical trials that demonstrate increased efficacy and fewer flares of disease, compared with placebo (Arthritis Rheum 2009;61:1168-78; Lancet 2011;377:721-31).

Although recent data regarding belimumab provide some hope, it "may be naive to think that a target-specific therapy exists in this complex, polygenic, heterogeneous disease," commented Dr. Kahn.

In concluding his talk, Dr. Kahn spoke about some barriers to the effective treatment of cSLE. These include physicians’ failure to detect the subtle changes of early disease, a lack of awareness regarding the neuropsychiatric manifestations of the disease, and physicians’ fear of being overly aggressive with treatment. He also warned about overt or covert adolescent noncompliance, and the danger of adolescents’ dropping out of the health care system once they need to transition to adult care.

NEW YORK – The good news in the management of childhood-onset systemic lupus erythematosus is that children who are diagnosed with the disease have a better prognosis and are living longer. The bad news is that over the course of a lifetime, affected children develop more morbidities and experience more medication-induced toxicities, which create additional management challenges, according to Dr. Phillip Kahn, who spoke at a meeting sponsored by New York University.

About 15%-20% of lupus cases present in childhood. Children younger than age 5 years rarely have the disease, but up to 40% of patients with childhood-onset SLE (cSLE) present prior to puberty, said Dr. Kahn of New York University. The incidence of cSLE is estimated to be 10-20 cases per 100,000 individuals, but the incidence is higher in blacks, at 200 cases per 100,000 individuals (Lupus 2007;16:546-9; 2005;14:83-8).

Often the diagnosis is delayed in children, as their clinical features do not fall within those defined by the 1997 American College of Rheumatology’s SLE criteria, which have not been validated in children. For example, Dr. Kahn described the case of a 13-year-old girl who presented with "constitutional symptoms" such as diffuse lymphadenopathy, malaise, weight loss, and fever – none of which are included in the 1997 criteria.

Mucocutaneous lesions are common in children, with up to 70% presenting with malar rash, 30% with alopecia, 66% with oral ulcers, and 10% with nasal ulcers. "Although nasal ulcers are less common in children than adults, they still may result in perforation of the septum," he said. Other common manifestations include painful polyarthritis, hepatosplenomegaly, headache, photosensitivity, and Raynaud’s phenomenon. Up to 40% of cSLE patients have neuropsychiatric symptoms, significantly more than do adults (Lupus 2008;17:314-22).

An important diagnostic tool for cSLE is a positive ANA (antinuclear antibody) finding, which is present in essentially all children with cSLE. In contrast, up to 10% of adults with SLE do not have a positive ANA, said Dr. Kahn.

Childhood-onset lupus may predict mortality in adults (Arthritis Care Res. [Hoboken] 2010;62:1152-9) and thus physicians must be vigilant about detecting, preventing, and aggressively treating some of its more dangerous manifestations. To prevent recurrent infection, Dr. Kahn recommends keeping a child up to date with immunizations, treating infections promptly when they occur, and considering PCP (pneumocystis carinii pneumonia) prophylaxis in the appropriate patient. Children with cSLE are also at increased risk for premature atherosclerosis, with dyslipidemia seen in 60%-85% of affected children (Nat. Clin. Pract. Rheumatol. 2008;4:258-65). Statin therapy may be appropriate in these children, especially to prevent long-term adverse events such as early MI. For example, adults – especially women – who had c SLE have increased incidence of MI when they reach their 30s (Arthritis Rheum. 2009;61:13-20). The ongoing APPLE (Atherosclerosis Prevention in Pediatric Lupus Erythematosus) trial is investigating, in greater detail, cardiovascular risk and the use of statins in cSLE (Lupus 2010;19:1315-25).

Because nephropathy is the main cause of mortality and morbidity in cSLE, it is important to monitor urinary markers of renal function. Nephropathy may take a greater toll on a pediatric patient, compared with adults; data show that children are more likely to have renal involvement, need hemodialysis and kidney transplantation, and require hospitalization and emergency department visits (Lupus 2008;17:314-22; Arthritis Rheum. 2009;61:13-20). To avoid end-stage renal disease, Dr. Kahn urges tight blood pressure control, as well as antihypertensive medication as necessary. "We know that 90% of the patients who ultimately develop renal disease will do so within the first 2 years of disease, so if you follow a patient for [more than 2] years and they do not develop the disease, the good news is they are much less likely to develop nephropathy," said Dr. Kahn.

In the 1950s, the 5-year survival rate for SLE in adults and children ranged from 18% to 70%; 30 years later, 5-year survival rose to 60%-93%, in large part because of the introduction of prednisone. Currently, prednisone is almost universally given to those with cSLE, and the proportion of children who are treated with prednisone is significantly higher than the proportion of adults with SLE (97% vs. 70%; P less than .0001) (Arthritis Rheum. 2008;58:556-62). The result is greater risk of osteoporosis, diabetes, and growth failure. In fact, up to 40% of cSLE patients have growth failure or short stature, in part because of chronic steroids.

That is why Dr. Kahn posed the question, "Can we move beyond steroids?" Until recently, the only Food and Drug Administration–approved medications for SLE were prednisone, hydroxychloroquine, and aspirin. Steroid-sparing immunomodulatory therapy is sometimes used off label when major organ (such as brain or kidney) involvement develops, but most evidence regarding the efficacy of these medications pertains to renal proliferative disease in adults.

Cyclophosphamide is often used for induction of nephritis, but 30% of patients may still develop end-stage renal disease despite treatment, said Dr. Kahn. Mycophenolate mofetil has been shown to have equal efficacy to cyclophosphamide in induction of remission of nephritis, but sufficient pediatric data are lacking. Azathioprine is effective for maintenance therapy of nephritis after the induction phase.

Other options that are sometimes used include intravenous immunoglobulin, methotrexate, calcineurin inhibitors (such as tacrolimus), plasmapheresis, and autologous stem cell transplantation/immunoablative therapy.

More recently, the focus has been on target-specific therapy for SLE with potentially fewer side effects. Rituximab, which targets B cells that have the CD20 surface antigen, is sometimes used to treat resistant patients, but there is no strong clinical evidence to demonstrate its effectiveness in cSLE (Arthritis Rheum. 2009;61:482-7; Clin. J. Am. Soc. Nephrol. 2009;4:579-87).

Abatacept, which targets T cells by binding to B7 peripheral membrane protein, has shown some clinical benefit in a phase IIB randomized clinical trial, and is undergoing clinical testing as an add-on induction therapy with cyclophosphamide (Arthritis Rheum. 2010;62:3077-87). Belimumab, a monoclonal antibody that targets BLyS (B-lymphocyte stimulator)/BAFF (B-cell activating factor), is the first FDA-approved medication for SLE in more than 50 years, based on recent clinical trials that demonstrate increased efficacy and fewer flares of disease, compared with placebo (Arthritis Rheum 2009;61:1168-78; Lancet 2011;377:721-31).

Although recent data regarding belimumab provide some hope, it "may be naive to think that a target-specific therapy exists in this complex, polygenic, heterogeneous disease," commented Dr. Kahn.

In concluding his talk, Dr. Kahn spoke about some barriers to the effective treatment of cSLE. These include physicians’ failure to detect the subtle changes of early disease, a lack of awareness regarding the neuropsychiatric manifestations of the disease, and physicians’ fear of being overly aggressive with treatment. He also warned about overt or covert adolescent noncompliance, and the danger of adolescents’ dropping out of the health care system once they need to transition to adult care.

NEW YORK – The good news in the management of childhood-onset systemic lupus erythematosus is that children who are diagnosed with the disease have a better prognosis and are living longer. The bad news is that over the course of a lifetime, affected children develop more morbidities and experience more medication-induced toxicities, which create additional management challenges, according to Dr. Phillip Kahn, who spoke at a meeting sponsored by New York University.

About 15%-20% of lupus cases present in childhood. Children younger than age 5 years rarely have the disease, but up to 40% of patients with childhood-onset SLE (cSLE) present prior to puberty, said Dr. Kahn of New York University. The incidence of cSLE is estimated to be 10-20 cases per 100,000 individuals, but the incidence is higher in blacks, at 200 cases per 100,000 individuals (Lupus 2007;16:546-9; 2005;14:83-8).

Often the diagnosis is delayed in children, as their clinical features do not fall within those defined by the 1997 American College of Rheumatology’s SLE criteria, which have not been validated in children. For example, Dr. Kahn described the case of a 13-year-old girl who presented with "constitutional symptoms" such as diffuse lymphadenopathy, malaise, weight loss, and fever – none of which are included in the 1997 criteria.

Mucocutaneous lesions are common in children, with up to 70% presenting with malar rash, 30% with alopecia, 66% with oral ulcers, and 10% with nasal ulcers. "Although nasal ulcers are less common in children than adults, they still may result in perforation of the septum," he said. Other common manifestations include painful polyarthritis, hepatosplenomegaly, headache, photosensitivity, and Raynaud’s phenomenon. Up to 40% of cSLE patients have neuropsychiatric symptoms, significantly more than do adults (Lupus 2008;17:314-22).

An important diagnostic tool for cSLE is a positive ANA (antinuclear antibody) finding, which is present in essentially all children with cSLE. In contrast, up to 10% of adults with SLE do not have a positive ANA, said Dr. Kahn.

Childhood-onset lupus may predict mortality in adults (Arthritis Care Res. [Hoboken] 2010;62:1152-9) and thus physicians must be vigilant about detecting, preventing, and aggressively treating some of its more dangerous manifestations. To prevent recurrent infection, Dr. Kahn recommends keeping a child up to date with immunizations, treating infections promptly when they occur, and considering PCP (pneumocystis carinii pneumonia) prophylaxis in the appropriate patient. Children with cSLE are also at increased risk for premature atherosclerosis, with dyslipidemia seen in 60%-85% of affected children (Nat. Clin. Pract. Rheumatol. 2008;4:258-65). Statin therapy may be appropriate in these children, especially to prevent long-term adverse events such as early MI. For example, adults – especially women – who had c SLE have increased incidence of MI when they reach their 30s (Arthritis Rheum. 2009;61:13-20). The ongoing APPLE (Atherosclerosis Prevention in Pediatric Lupus Erythematosus) trial is investigating, in greater detail, cardiovascular risk and the use of statins in cSLE (Lupus 2010;19:1315-25).

Because nephropathy is the main cause of mortality and morbidity in cSLE, it is important to monitor urinary markers of renal function. Nephropathy may take a greater toll on a pediatric patient, compared with adults; data show that children are more likely to have renal involvement, need hemodialysis and kidney transplantation, and require hospitalization and emergency department visits (Lupus 2008;17:314-22; Arthritis Rheum. 2009;61:13-20). To avoid end-stage renal disease, Dr. Kahn urges tight blood pressure control, as well as antihypertensive medication as necessary. "We know that 90% of the patients who ultimately develop renal disease will do so within the first 2 years of disease, so if you follow a patient for [more than 2] years and they do not develop the disease, the good news is they are much less likely to develop nephropathy," said Dr. Kahn.

In the 1950s, the 5-year survival rate for SLE in adults and children ranged from 18% to 70%; 30 years later, 5-year survival rose to 60%-93%, in large part because of the introduction of prednisone. Currently, prednisone is almost universally given to those with cSLE, and the proportion of children who are treated with prednisone is significantly higher than the proportion of adults with SLE (97% vs. 70%; P less than .0001) (Arthritis Rheum. 2008;58:556-62). The result is greater risk of osteoporosis, diabetes, and growth failure. In fact, up to 40% of cSLE patients have growth failure or short stature, in part because of chronic steroids.

That is why Dr. Kahn posed the question, "Can we move beyond steroids?" Until recently, the only Food and Drug Administration–approved medications for SLE were prednisone, hydroxychloroquine, and aspirin. Steroid-sparing immunomodulatory therapy is sometimes used off label when major organ (such as brain or kidney) involvement develops, but most evidence regarding the efficacy of these medications pertains to renal proliferative disease in adults.

Cyclophosphamide is often used for induction of nephritis, but 30% of patients may still develop end-stage renal disease despite treatment, said Dr. Kahn. Mycophenolate mofetil has been shown to have equal efficacy to cyclophosphamide in induction of remission of nephritis, but sufficient pediatric data are lacking. Azathioprine is effective for maintenance therapy of nephritis after the induction phase.

Other options that are sometimes used include intravenous immunoglobulin, methotrexate, calcineurin inhibitors (such as tacrolimus), plasmapheresis, and autologous stem cell transplantation/immunoablative therapy.

More recently, the focus has been on target-specific therapy for SLE with potentially fewer side effects. Rituximab, which targets B cells that have the CD20 surface antigen, is sometimes used to treat resistant patients, but there is no strong clinical evidence to demonstrate its effectiveness in cSLE (Arthritis Rheum. 2009;61:482-7; Clin. J. Am. Soc. Nephrol. 2009;4:579-87).

Abatacept, which targets T cells by binding to B7 peripheral membrane protein, has shown some clinical benefit in a phase IIB randomized clinical trial, and is undergoing clinical testing as an add-on induction therapy with cyclophosphamide (Arthritis Rheum. 2010;62:3077-87). Belimumab, a monoclonal antibody that targets BLyS (B-lymphocyte stimulator)/BAFF (B-cell activating factor), is the first FDA-approved medication for SLE in more than 50 years, based on recent clinical trials that demonstrate increased efficacy and fewer flares of disease, compared with placebo (Arthritis Rheum 2009;61:1168-78; Lancet 2011;377:721-31).

Although recent data regarding belimumab provide some hope, it "may be naive to think that a target-specific therapy exists in this complex, polygenic, heterogeneous disease," commented Dr. Kahn.

In concluding his talk, Dr. Kahn spoke about some barriers to the effective treatment of cSLE. These include physicians’ failure to detect the subtle changes of early disease, a lack of awareness regarding the neuropsychiatric manifestations of the disease, and physicians’ fear of being overly aggressive with treatment. He also warned about overt or covert adolescent noncompliance, and the danger of adolescents’ dropping out of the health care system once they need to transition to adult care.

NEW YORK – Tumor necrosis factor inhibitors reduced the risk of myocardial infarction, stroke and cardiovascular-related death in patients with rheumatoid arthritis, according to Dr. Jeffrey D. Greenberg, who presented the findings at a rheumatology meeting sponsored by New York University. Methotrexate was not associated with a reduced risk of cardiovascular events, and prednisone use was associated with a dose-dependent increased risk.

The findings come from CORRONA (Consortium of Rheumatology Researchers of North America), a registry of patients with RA or psoriatic arthritis. The study population involved 10,156 patients followed for a median of 23 months. A subset analysis was performed, and 88 patients were identified who had experienced nonfatal myocardial infarction (n = 26), transient ischemic attack or stroke (n = 45), or cardiovascular-related death (n = 17) (Ann. Rheum. Dis. 2011;70:576-82).

In an editorial accompanying Dr. Greenberg’s report (Ann. Rheum. Dis. 2011 70:561-2), Dr. Johan Askling of Karolinska University Hospital, Stockholm, and Dr. Will Dixon of the University of Manchester (England) praiseed Dr. Greenberg’s study in terms of its methodology and transparency. However, they point out that other studies, such as those published by the British Society for Rheumatology Biologics Register (BSRBR), have not supported a cardiovascular protective effect for TNF inhibitors.

Nonbiologic disease-modifying antirheumatic drugs (DMARDs) other than methotrexate served as the reference cohort for all therapeutic comparisons. Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduction in risk (HR 0.83, 95% CI 0.44-1.57). In contrast, patients using a TNF inhibitor had a 61% reduced risk of the primary composite cardiovascular end point (HR 0.39, 0.18-0.74).

Dr. Greenberg also showed significant reductions in risk for those taking TNF inhibitors compared with those taking nonbiological DMARDs as measured by a composite end point that included CV deaths (HR = 0.39, 0.19-0.82) and a composite end point that excluded CV deaths (HR = 0.35, 0.16-0.74), and nonfatal MI (HR = 0.24, 0.06-0.95). There was a trend toward reduced risk for nonfatal TIA/stroke (HR = 0.44, 0.18-1.09). "These data indicate that TNF antagonists may represent a therapeutic strategy to attenuate the heightened cardiovascular risk experienced by RA patients," Dr. Greenberg said.

"As expected, there was a dose-dependent increased risk of cardiovascular events with steroids," said Dr. Greenberg of New York University. Prednisone doses between 1-2.5 mg and 3-7 mg almost doubled the risk (HR 1.90 and 1.89, respectively) while doses greater than 7 mg tripled the risk (HR 3.00, 1.44-6.25) (P = .04).

Dr. Greenberg advocates a three-step strategy to decrease cardiovascular risk in RA.

The first is to adhere to recommended cardiovascular disease prevention, screening, and guidelines. This includes management of "traditional" cardiovascular risk factors such as blood pressure, diabetes, lipids, smoking, and obesity, he said.

The second component – as supported by his study’s results – is to minimize use of NSAIDs and corticosteroids, and to utilize steroid-sparing options if possible.

The third component is to aim for tighter control of RA disease activity. For patients at increased CV risk, "I would treat RA aggressively," said Dr. Greenberg. Even so, he acknowledged that it has not been proved that the strategy would alter the natural course of developing cardiovascular disease. It remains to be determined what measures should be targeted, he said, suggesting options such as a specific disease activity score, C-reactive protein levels, or other inflammatory biomarkers.

Indeed, the lack of CV risk assessment tools and evidence-based practice guidelines for improving cardiovascular risk management in RA patients is getting a lot of attention.

Dr. Sherine E. Gabriel and researcher Cynthia S. Crowson of the Mayo Clinic, Rochester, Minn., discussed the limitations of both the SCORE and Framingham Risk Scores (Ann. Rheum. Dis. 2011;70:719-21). While applauding the European League Against Rheumatism (EULAR) for formulating guidelines to improve cardiovascular management in patients with RA, the researchers point out several areas of weakness. Cardiovascular disease risk, for example, is not necessarily associated with longer disease duration. In fact, studies show there is an increased risk of cardiovascular events early in the course of RA, they noted. The risks also are unclear for patients who are RF negative or ACPA negative. Further, RA patients may respond atypically to traditional cardiovascular risk factors.

Dr. Greenberg has done consulting for Centocor, CORRONA, Novartis Pharmaceutical Corp, Roche Laboratories, and UCB. He has received research and grant support from the Arthritis Foundation and the National Institutes of Health. He receives consulting fees from, or owns stock shares in, AstraZeneca, CORRONA, Genentech, Novartis, and Pfizer.

NEW YORK – Tumor necrosis factor inhibitors reduced the risk of myocardial infarction, stroke and cardiovascular-related death in patients with rheumatoid arthritis, according to Dr. Jeffrey D. Greenberg, who presented the findings at a rheumatology meeting sponsored by New York University. Methotrexate was not associated with a reduced risk of cardiovascular events, and prednisone use was associated with a dose-dependent increased risk.

The findings come from CORRONA (Consortium of Rheumatology Researchers of North America), a registry of patients with RA or psoriatic arthritis. The study population involved 10,156 patients followed for a median of 23 months. A subset analysis was performed, and 88 patients were identified who had experienced nonfatal myocardial infarction (n = 26), transient ischemic attack or stroke (n = 45), or cardiovascular-related death (n = 17) (Ann. Rheum. Dis. 2011;70:576-82).

In an editorial accompanying Dr. Greenberg’s report (Ann. Rheum. Dis. 2011 70:561-2), Dr. Johan Askling of Karolinska University Hospital, Stockholm, and Dr. Will Dixon of the University of Manchester (England) praiseed Dr. Greenberg’s study in terms of its methodology and transparency. However, they point out that other studies, such as those published by the British Society for Rheumatology Biologics Register (BSRBR), have not supported a cardiovascular protective effect for TNF inhibitors.

Nonbiologic disease-modifying antirheumatic drugs (DMARDs) other than methotrexate served as the reference cohort for all therapeutic comparisons. Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduction in risk (HR 0.83, 95% CI 0.44-1.57). In contrast, patients using a TNF inhibitor had a 61% reduced risk of the primary composite cardiovascular end point (HR 0.39, 0.18-0.74).

Dr. Greenberg also showed significant reductions in risk for those taking TNF inhibitors compared with those taking nonbiological DMARDs as measured by a composite end point that included CV deaths (HR = 0.39, 0.19-0.82) and a composite end point that excluded CV deaths (HR = 0.35, 0.16-0.74), and nonfatal MI (HR = 0.24, 0.06-0.95). There was a trend toward reduced risk for nonfatal TIA/stroke (HR = 0.44, 0.18-1.09). "These data indicate that TNF antagonists may represent a therapeutic strategy to attenuate the heightened cardiovascular risk experienced by RA patients," Dr. Greenberg said.

"As expected, there was a dose-dependent increased risk of cardiovascular events with steroids," said Dr. Greenberg of New York University. Prednisone doses between 1-2.5 mg and 3-7 mg almost doubled the risk (HR 1.90 and 1.89, respectively) while doses greater than 7 mg tripled the risk (HR 3.00, 1.44-6.25) (P = .04).

Dr. Greenberg advocates a three-step strategy to decrease cardiovascular risk in RA.

The first is to adhere to recommended cardiovascular disease prevention, screening, and guidelines. This includes management of "traditional" cardiovascular risk factors such as blood pressure, diabetes, lipids, smoking, and obesity, he said.

The second component – as supported by his study’s results – is to minimize use of NSAIDs and corticosteroids, and to utilize steroid-sparing options if possible.

The third component is to aim for tighter control of RA disease activity. For patients at increased CV risk, "I would treat RA aggressively," said Dr. Greenberg. Even so, he acknowledged that it has not been proved that the strategy would alter the natural course of developing cardiovascular disease. It remains to be determined what measures should be targeted, he said, suggesting options such as a specific disease activity score, C-reactive protein levels, or other inflammatory biomarkers.

Indeed, the lack of CV risk assessment tools and evidence-based practice guidelines for improving cardiovascular risk management in RA patients is getting a lot of attention.

Dr. Sherine E. Gabriel and researcher Cynthia S. Crowson of the Mayo Clinic, Rochester, Minn., discussed the limitations of both the SCORE and Framingham Risk Scores (Ann. Rheum. Dis. 2011;70:719-21). While applauding the European League Against Rheumatism (EULAR) for formulating guidelines to improve cardiovascular management in patients with RA, the researchers point out several areas of weakness. Cardiovascular disease risk, for example, is not necessarily associated with longer disease duration. In fact, studies show there is an increased risk of cardiovascular events early in the course of RA, they noted. The risks also are unclear for patients who are RF negative or ACPA negative. Further, RA patients may respond atypically to traditional cardiovascular risk factors.

Dr. Greenberg has done consulting for Centocor, CORRONA, Novartis Pharmaceutical Corp, Roche Laboratories, and UCB. He has received research and grant support from the Arthritis Foundation and the National Institutes of Health. He receives consulting fees from, or owns stock shares in, AstraZeneca, CORRONA, Genentech, Novartis, and Pfizer.

NEW YORK – Tumor necrosis factor inhibitors reduced the risk of myocardial infarction, stroke and cardiovascular-related death in patients with rheumatoid arthritis, according to Dr. Jeffrey D. Greenberg, who presented the findings at a rheumatology meeting sponsored by New York University. Methotrexate was not associated with a reduced risk of cardiovascular events, and prednisone use was associated with a dose-dependent increased risk.

The findings come from CORRONA (Consortium of Rheumatology Researchers of North America), a registry of patients with RA or psoriatic arthritis. The study population involved 10,156 patients followed for a median of 23 months. A subset analysis was performed, and 88 patients were identified who had experienced nonfatal myocardial infarction (n = 26), transient ischemic attack or stroke (n = 45), or cardiovascular-related death (n = 17) (Ann. Rheum. Dis. 2011;70:576-82).

In an editorial accompanying Dr. Greenberg’s report (Ann. Rheum. Dis. 2011 70:561-2), Dr. Johan Askling of Karolinska University Hospital, Stockholm, and Dr. Will Dixon of the University of Manchester (England) praiseed Dr. Greenberg’s study in terms of its methodology and transparency. However, they point out that other studies, such as those published by the British Society for Rheumatology Biologics Register (BSRBR), have not supported a cardiovascular protective effect for TNF inhibitors.

Nonbiologic disease-modifying antirheumatic drugs (DMARDs) other than methotrexate served as the reference cohort for all therapeutic comparisons. Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduction in risk (HR 0.83, 95% CI 0.44-1.57). In contrast, patients using a TNF inhibitor had a 61% reduced risk of the primary composite cardiovascular end point (HR 0.39, 0.18-0.74).

Dr. Greenberg also showed significant reductions in risk for those taking TNF inhibitors compared with those taking nonbiological DMARDs as measured by a composite end point that included CV deaths (HR = 0.39, 0.19-0.82) and a composite end point that excluded CV deaths (HR = 0.35, 0.16-0.74), and nonfatal MI (HR = 0.24, 0.06-0.95). There was a trend toward reduced risk for nonfatal TIA/stroke (HR = 0.44, 0.18-1.09). "These data indicate that TNF antagonists may represent a therapeutic strategy to attenuate the heightened cardiovascular risk experienced by RA patients," Dr. Greenberg said.

"As expected, there was a dose-dependent increased risk of cardiovascular events with steroids," said Dr. Greenberg of New York University. Prednisone doses between 1-2.5 mg and 3-7 mg almost doubled the risk (HR 1.90 and 1.89, respectively) while doses greater than 7 mg tripled the risk (HR 3.00, 1.44-6.25) (P = .04).

Dr. Greenberg advocates a three-step strategy to decrease cardiovascular risk in RA.

The first is to adhere to recommended cardiovascular disease prevention, screening, and guidelines. This includes management of "traditional" cardiovascular risk factors such as blood pressure, diabetes, lipids, smoking, and obesity, he said.

The second component – as supported by his study’s results – is to minimize use of NSAIDs and corticosteroids, and to utilize steroid-sparing options if possible.

The third component is to aim for tighter control of RA disease activity. For patients at increased CV risk, "I would treat RA aggressively," said Dr. Greenberg. Even so, he acknowledged that it has not been proved that the strategy would alter the natural course of developing cardiovascular disease. It remains to be determined what measures should be targeted, he said, suggesting options such as a specific disease activity score, C-reactive protein levels, or other inflammatory biomarkers.

Indeed, the lack of CV risk assessment tools and evidence-based practice guidelines for improving cardiovascular risk management in RA patients is getting a lot of attention.

Dr. Sherine E. Gabriel and researcher Cynthia S. Crowson of the Mayo Clinic, Rochester, Minn., discussed the limitations of both the SCORE and Framingham Risk Scores (Ann. Rheum. Dis. 2011;70:719-21). While applauding the European League Against Rheumatism (EULAR) for formulating guidelines to improve cardiovascular management in patients with RA, the researchers point out several areas of weakness. Cardiovascular disease risk, for example, is not necessarily associated with longer disease duration. In fact, studies show there is an increased risk of cardiovascular events early in the course of RA, they noted. The risks also are unclear for patients who are RF negative or ACPA negative. Further, RA patients may respond atypically to traditional cardiovascular risk factors.

Dr. Greenberg has done consulting for Centocor, CORRONA, Novartis Pharmaceutical Corp, Roche Laboratories, and UCB. He has received research and grant support from the Arthritis Foundation and the National Institutes of Health. He receives consulting fees from, or owns stock shares in, AstraZeneca, CORRONA, Genentech, Novartis, and Pfizer.

NEW YORK – Tumor necrosis factor inhibitors reduced the risk of myocardial infarction, stroke and cardiovascular-related death in patients with rheumatoid arthritis, according to Dr. Jeffrey D. Greenberg, who presented the findings at a rheumatology meeting sponsored by New York University. Methotrexate was not associated with a reduced risk of cardiovascular events, and prednisone use was associated with a dose-dependent increased risk.

The findings come from CORRONA (Consortium of Rheumatology Researchers of North America), a registry of patients with RA or psoriatic arthritis. The study population involved 10,156 patients followed for a median of 23 months. A subset analysis was performed, and 88 patients were identified who had experienced nonfatal myocardial infarction (n = 26), transient ischemic attack or stroke (n = 45), or cardiovascular-related death (n = 17) (Ann. Rheum. Dis. 2011;70:576-82).

In an editorial accompanying Dr. Greenberg’s report (Ann. Rheum. Dis. 2011 70:561-2), Dr. Johan Askling of Karolinska University Hospital, Stockholm, and Dr. Will Dixon of the University of Manchester (England) praiseed Dr. Greenberg’s study in terms of its methodology and transparency. However, they point out that other studies, such as those published by the British Society for Rheumatology Biologics Register (BSRBR), have not supported a cardiovascular protective effect for TNF inhibitors.

Nonbiologic disease-modifying antirheumatic drugs (DMARDs) other than methotrexate served as the reference cohort for all therapeutic comparisons. Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduction in risk (HR 0.83, 95% CI 0.44-1.57). In contrast, patients using a TNF inhibitor had a 61% reduced risk of the primary composite cardiovascular end point (HR 0.39, 0.18-0.74).

Dr. Greenberg also showed significant reductions in risk for those taking TNF inhibitors compared with those taking nonbiological DMARDs as measured by a composite end point that included CV deaths (HR = 0.39, 0.19-0.82) and a composite end point that excluded CV deaths (HR = 0.35, 0.16-0.74), and nonfatal MI (HR = 0.24, 0.06-0.95). There was a trend toward reduced risk for nonfatal TIA/stroke (HR = 0.44, 0.18-1.09). "These data indicate that TNF antagonists may represent a therapeutic strategy to attenuate the heightened cardiovascular risk experienced by RA patients," Dr. Greenberg said.

"As expected, there was a dose-dependent increased risk of cardiovascular events with steroids," said Dr. Greenberg of New York University. Prednisone doses between 1-2.5 mg and 3-7 mg almost doubled the risk (HR 1.90 and 1.89, respectively) while doses greater than 7 mg tripled the risk (HR 3.00, 1.44-6.25) (P = .04).

Dr. Greenberg advocates a three-step strategy to decrease cardiovascular risk in RA.

The first is to adhere to recommended cardiovascular disease prevention, screening, and guidelines. This includes management of "traditional" cardiovascular risk factors such as blood pressure, diabetes, lipids, smoking, and obesity, he said.

The second component – as supported by his study’s results – is to minimize use of NSAIDs and corticosteroids, and to utilize steroid-sparing options if possible.

The third component is to aim for tighter control of RA disease activity. For patients at increased CV risk, "I would treat RA aggressively," said Dr. Greenberg. Even so, he acknowledged that it has not been proved that the strategy would alter the natural course of developing cardiovascular disease. It remains to be determined what measures should be targeted, he said, suggesting options such as a specific disease activity score, C-reactive protein levels, or other inflammatory biomarkers.

Indeed, the lack of CV risk assessment tools and evidence-based practice guidelines for improving cardiovascular risk management in RA patients is getting a lot of attention.

Dr. Sherine E. Gabriel and researcher Cynthia S. Crowson of the Mayo Clinic, Rochester, Minn., discussed the limitations of both the SCORE and Framingham Risk Scores (Ann. Rheum. Dis. 2011;70:719-21). While applauding the European League Against Rheumatism (EULAR) for formulating guidelines to improve cardiovascular management in patients with RA, the researchers point out several areas of weakness. Cardiovascular disease risk, for example, is not necessarily associated with longer disease duration. In fact, studies show there is an increased risk of cardiovascular events early in the course of RA, they noted. The risks also are unclear for patients who are RF negative or ACPA negative. Further, RA patients may respond atypically to traditional cardiovascular risk factors.

Dr. Greenberg has done consulting for Centocor, CORRONA, Novartis Pharmaceutical Corp, Roche Laboratories, and UCB. He has received research and grant support from the Arthritis Foundation and the National Institutes of Health. He receives consulting fees from, or owns stock shares in, AstraZeneca, CORRONA, Genentech, Novartis, and Pfizer.

NEW YORK – Tumor necrosis factor inhibitors reduced the risk of myocardial infarction, stroke and cardiovascular-related death in patients with rheumatoid arthritis, according to Dr. Jeffrey D. Greenberg, who presented the findings at a rheumatology meeting sponsored by New York University. Methotrexate was not associated with a reduced risk of cardiovascular events, and prednisone use was associated with a dose-dependent increased risk.

The findings come from CORRONA (Consortium of Rheumatology Researchers of North America), a registry of patients with RA or psoriatic arthritis. The study population involved 10,156 patients followed for a median of 23 months. A subset analysis was performed, and 88 patients were identified who had experienced nonfatal myocardial infarction (n = 26), transient ischemic attack or stroke (n = 45), or cardiovascular-related death (n = 17) (Ann. Rheum. Dis. 2011;70:576-82).

In an editorial accompanying Dr. Greenberg’s report (Ann. Rheum. Dis. 2011 70:561-2), Dr. Johan Askling of Karolinska University Hospital, Stockholm, and Dr. Will Dixon of the University of Manchester (England) praiseed Dr. Greenberg’s study in terms of its methodology and transparency. However, they point out that other studies, such as those published by the British Society for Rheumatology Biologics Register (BSRBR), have not supported a cardiovascular protective effect for TNF inhibitors.

Nonbiologic disease-modifying antirheumatic drugs (DMARDs) other than methotrexate served as the reference cohort for all therapeutic comparisons. Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduction in risk (HR 0.83, 95% CI 0.44-1.57). In contrast, patients using a TNF inhibitor had a 61% reduced risk of the primary composite cardiovascular end point (HR 0.39, 0.18-0.74).

Dr. Greenberg also showed significant reductions in risk for those taking TNF inhibitors compared with those taking nonbiological DMARDs as measured by a composite end point that included CV deaths (HR = 0.39, 0.19-0.82) and a composite end point that excluded CV deaths (HR = 0.35, 0.16-0.74), and nonfatal MI (HR = 0.24, 0.06-0.95). There was a trend toward reduced risk for nonfatal TIA/stroke (HR = 0.44, 0.18-1.09). "These data indicate that TNF antagonists may represent a therapeutic strategy to attenuate the heightened cardiovascular risk experienced by RA patients," Dr. Greenberg said.

"As expected, there was a dose-dependent increased risk of cardiovascular events with steroids," said Dr. Greenberg of New York University. Prednisone doses between 1-2.5 mg and 3-7 mg almost doubled the risk (HR 1.90 and 1.89, respectively) while doses greater than 7 mg tripled the risk (HR 3.00, 1.44-6.25) (P = .04).

Dr. Greenberg advocates a three-step strategy to decrease cardiovascular risk in RA.

The first is to adhere to recommended cardiovascular disease prevention, screening, and guidelines. This includes management of "traditional" cardiovascular risk factors such as blood pressure, diabetes, lipids, smoking, and obesity, he said.

The second component – as supported by his study’s results – is to minimize use of NSAIDs and corticosteroids, and to utilize steroid-sparing options if possible.

The third component is to aim for tighter control of RA disease activity. For patients at increased CV risk, "I would treat RA aggressively," said Dr. Greenberg. Even so, he acknowledged that it has not been proved that the strategy would alter the natural course of developing cardiovascular disease. It remains to be determined what measures should be targeted, he said, suggesting options such as a specific disease activity score, C-reactive protein levels, or other inflammatory biomarkers.

Indeed, the lack of CV risk assessment tools and evidence-based practice guidelines for improving cardiovascular risk management in RA patients is getting a lot of attention.

Dr. Sherine E. Gabriel and researcher Cynthia S. Crowson of the Mayo Clinic, Rochester, Minn., discussed the limitations of both the SCORE and Framingham Risk Scores (Ann. Rheum. Dis. 2011;70:719-21). While applauding the European League Against Rheumatism (EULAR) for formulating guidelines to improve cardiovascular management in patients with RA, the researchers point out several areas of weakness. Cardiovascular disease risk, for example, is not necessarily associated with longer disease duration. In fact, studies show there is an increased risk of cardiovascular events early in the course of RA, they noted. The risks also are unclear for patients who are RF negative or ACPA negative. Further, RA patients may respond atypically to traditional cardiovascular risk factors.

Dr. Greenberg has done consulting for Centocor, CORRONA, Novartis Pharmaceutical Corp, Roche Laboratories, and UCB. He has received research and grant support from the Arthritis Foundation and the National Institutes of Health. He receives consulting fees from, or owns stock shares in, AstraZeneca, CORRONA, Genentech, Novartis, and Pfizer.

NEW YORK – Tumor necrosis factor inhibitors reduced the risk of myocardial infarction, stroke and cardiovascular-related death in patients with rheumatoid arthritis, according to Dr. Jeffrey D. Greenberg, who presented the findings at a rheumatology meeting sponsored by New York University. Methotrexate was not associated with a reduced risk of cardiovascular events, and prednisone use was associated with a dose-dependent increased risk.

The findings come from CORRONA (Consortium of Rheumatology Researchers of North America), a registry of patients with RA or psoriatic arthritis. The study population involved 10,156 patients followed for a median of 23 months. A subset analysis was performed, and 88 patients were identified who had experienced nonfatal myocardial infarction (n = 26), transient ischemic attack or stroke (n = 45), or cardiovascular-related death (n = 17) (Ann. Rheum. Dis. 2011;70:576-82).

In an editorial accompanying Dr. Greenberg’s report (Ann. Rheum. Dis. 2011 70:561-2), Dr. Johan Askling of Karolinska University Hospital, Stockholm, and Dr. Will Dixon of the University of Manchester (England) praiseed Dr. Greenberg’s study in terms of its methodology and transparency. However, they point out that other studies, such as those published by the British Society for Rheumatology Biologics Register (BSRBR), have not supported a cardiovascular protective effect for TNF inhibitors.

Nonbiologic disease-modifying antirheumatic drugs (DMARDs) other than methotrexate served as the reference cohort for all therapeutic comparisons. Relative to nonbiologic DMARDs, methotrexate use was associated with a nonsignificant reduction in risk (HR 0.83, 95% CI 0.44-1.57). In contrast, patients using a TNF inhibitor had a 61% reduced risk of the primary composite cardiovascular end point (HR 0.39, 0.18-0.74).

Dr. Greenberg also showed significant reductions in risk for those taking TNF inhibitors compared with those taking nonbiological DMARDs as measured by a composite end point that included CV deaths (HR = 0.39, 0.19-0.82) and a composite end point that excluded CV deaths (HR = 0.35, 0.16-0.74), and nonfatal MI (HR = 0.24, 0.06-0.95). There was a trend toward reduced risk for nonfatal TIA/stroke (HR = 0.44, 0.18-1.09). "These data indicate that TNF antagonists may represent a therapeutic strategy to attenuate the heightened cardiovascular risk experienced by RA patients," Dr. Greenberg said.

"As expected, there was a dose-dependent increased risk of cardiovascular events with steroids," said Dr. Greenberg of New York University. Prednisone doses between 1-2.5 mg and 3-7 mg almost doubled the risk (HR 1.90 and 1.89, respectively) while doses greater than 7 mg tripled the risk (HR 3.00, 1.44-6.25) (P = .04).

Dr. Greenberg advocates a three-step strategy to decrease cardiovascular risk in RA.

The first is to adhere to recommended cardiovascular disease prevention, screening, and guidelines. This includes management of "traditional" cardiovascular risk factors such as blood pressure, diabetes, lipids, smoking, and obesity, he said.

The second component – as supported by his study’s results – is to minimize use of NSAIDs and corticosteroids, and to utilize steroid-sparing options if possible.

The third component is to aim for tighter control of RA disease activity. For patients at increased CV risk, "I would treat RA aggressively," said Dr. Greenberg. Even so, he acknowledged that it has not been proved that the strategy would alter the natural course of developing cardiovascular disease. It remains to be determined what measures should be targeted, he said, suggesting options such as a specific disease activity score, C-reactive protein levels, or other inflammatory biomarkers.

Indeed, the lack of CV risk assessment tools and evidence-based practice guidelines for improving cardiovascular risk management in RA patients is getting a lot of attention.

Dr. Sherine E. Gabriel and researcher Cynthia S. Crowson of the Mayo Clinic, Rochester, Minn., discussed the limitations of both the SCORE and Framingham Risk Scores (Ann. Rheum. Dis. 2011;70:719-21). While applauding the European League Against Rheumatism (EULAR) for formulating guidelines to improve cardiovascular management in patients with RA, the researchers point out several areas of weakness. Cardiovascular disease risk, for example, is not necessarily associated with longer disease duration. In fact, studies show there is an increased risk of cardiovascular events early in the course of RA, they noted. The risks also are unclear for patients who are RF negative or ACPA negative. Further, RA patients may respond atypically to traditional cardiovascular risk factors.

Dr. Greenberg has done consulting for Centocor, CORRONA, Novartis Pharmaceutical Corp, Roche Laboratories, and UCB. He has received research and grant support from the Arthritis Foundation and the National Institutes of Health. He receives consulting fees from, or owns stock shares in, AstraZeneca, CORRONA, Genentech, Novartis, and Pfizer.

NEW YORK – When magnetic resonance imaging is used instead of plain x-rays in patients with early inflammatory back pain, the diagnostic accuracy for spondyloarthritis jumps from 25% to 70%, according to Dr. Maxime Dougados, who spoke at a rheumatology meeting sponsored by New York University.

"Seventy-five percent of the time, you cannot make the diagnosis with plain x-rays," said Dr. Dougados, professor of rheumatology at Paris-Descartes University/Cochin Hospital in Paris and the president-elect of EULAR. He presented the Ira Goldstein Memorial Lecture at the meeting, focusing on spondyloarthritis (SpA).

Dr. Dougados presented the as yet unpublished results from the DESIR cohort, a large French national multicenter database of long-term follow-up of 708 patients presenting with early inflammatory back pain that was initiated by the French Society of Rheumatology. Patients were recruited between December 2007 and April 2010 if they had inflammatory back pain lasting more than 3 months and less than 3 years. The group will be followed for 10 years in the ongoing study. At baseline, the mean age was 35 years, 54% were female and 57% were HLA-B27 positive (Joint Bone Spine 2011 March 30 [doi: 10.1016/j.jbspin.2011.01.013]).

Looking at radiological sacroiliac changes, the diagnosis was "obvious" for 25.6% of the cohort, "doubtful" for 21.3%, and "normal" for 53.1%. "These results indicate that at the first clinical visit, the interview is very important to pick up other clinical symptoms," said Dr. Dougados. In fact, about 80% were found to have non-axial clinical manifestations, including articular peripheral involvement, enthesopathy, dactylitis, anterior chest wall pain, uveitis, or psoriasis.

Using MRI, 70% of the cohort were determined to have "obvious" sacroiliitis, about 20% had a "doubtful" diagnosis and about 10% were thought to be "normal."

"These results indicate that you can detect early abnormalities of the sacroiliac joint on MRI even if x-rays are normal," he said.

According to Dr. Dougados, these imaging findings fit well with recent results from the DECLIC study, in which 163 rheumatologists were asked to diagnose 472 patients with early inflammatory back pain, including 161 patients with spondyloarthritis, according to four different sets of criteria. The specificity of the modified New York criteria, which relies on radiographic signs of sacroiliitis (unilateral grade III or bilateral grade II), fell well below that of the modified Amor criteria, the modified ESSG (European Spondyloarthropathy Study Group) criteria, and the ASAS (Assessment of Spondyloarthritis International Society) criteria. The latter three criteria include the option of diagnosing sacroiliitis with MRI.

In the new classification criteria from the ASAS, separate criteria are listed for patients with axial SpA with and without peripheral manifestations and patients with peripheral manifestations only. For axial SpA, one diagnostic pathway requires sacroiliitis on imaging plus one or more SpA feature. Sacroiliitis on MRI is given as much weight as is sacroiliitis on radiographs (Best Pract. Res. Clin. Rheumatol. 2010;24:589-604). The other pathway requires HLA-B27 positivity plus two or more SpA features. In patients with peripheral manifestations only, the requirements include peripheral arthritis, enthesitis or dactylitis plus one or more SpA features, including sacroiliitis on imaging.

Dr. Dougados also spoke about recent findings showing that patients with SpA were more likely to have distinct noninflammatory spinal MRI lesions (known as Fatty Romanus lesions) than were patients with degenerative arthritis or spinal malignancy (Ann. Rheum. Dis. 2010; 69:891-94).

"As MRI is becoming more important, rheumatologists should be trained to interpret MRIs," he said. "You don’t need to be a specialist in radiology."

Dr. Dougados has received grants for research projects and/or honorarium fees for participation at advisory boards/symposiums from Abbott, Bristol-Myers Squibb, Merck, Pfizer, Sanofi, and UCB.

NEW YORK – When magnetic resonance imaging is used instead of plain x-rays in patients with early inflammatory back pain, the diagnostic accuracy for spondyloarthritis jumps from 25% to 70%, according to Dr. Maxime Dougados, who spoke at a rheumatology meeting sponsored by New York University.

"Seventy-five percent of the time, you cannot make the diagnosis with plain x-rays," said Dr. Dougados, professor of rheumatology at Paris-Descartes University/Cochin Hospital in Paris and the president-elect of EULAR. He presented the Ira Goldstein Memorial Lecture at the meeting, focusing on spondyloarthritis (SpA).

Dr. Dougados presented the as yet unpublished results from the DESIR cohort, a large French national multicenter database of long-term follow-up of 708 patients presenting with early inflammatory back pain that was initiated by the French Society of Rheumatology. Patients were recruited between December 2007 and April 2010 if they had inflammatory back pain lasting more than 3 months and less than 3 years. The group will be followed for 10 years in the ongoing study. At baseline, the mean age was 35 years, 54% were female and 57% were HLA-B27 positive (Joint Bone Spine 2011 March 30 [doi: 10.1016/j.jbspin.2011.01.013]).

Looking at radiological sacroiliac changes, the diagnosis was "obvious" for 25.6% of the cohort, "doubtful" for 21.3%, and "normal" for 53.1%. "These results indicate that at the first clinical visit, the interview is very important to pick up other clinical symptoms," said Dr. Dougados. In fact, about 80% were found to have non-axial clinical manifestations, including articular peripheral involvement, enthesopathy, dactylitis, anterior chest wall pain, uveitis, or psoriasis.

Using MRI, 70% of the cohort were determined to have "obvious" sacroiliitis, about 20% had a "doubtful" diagnosis and about 10% were thought to be "normal."

"These results indicate that you can detect early abnormalities of the sacroiliac joint on MRI even if x-rays are normal," he said.

According to Dr. Dougados, these imaging findings fit well with recent results from the DECLIC study, in which 163 rheumatologists were asked to diagnose 472 patients with early inflammatory back pain, including 161 patients with spondyloarthritis, according to four different sets of criteria. The specificity of the modified New York criteria, which relies on radiographic signs of sacroiliitis (unilateral grade III or bilateral grade II), fell well below that of the modified Amor criteria, the modified ESSG (European Spondyloarthropathy Study Group) criteria, and the ASAS (Assessment of Spondyloarthritis International Society) criteria. The latter three criteria include the option of diagnosing sacroiliitis with MRI.

In the new classification criteria from the ASAS, separate criteria are listed for patients with axial SpA with and without peripheral manifestations and patients with peripheral manifestations only. For axial SpA, one diagnostic pathway requires sacroiliitis on imaging plus one or more SpA feature. Sacroiliitis on MRI is given as much weight as is sacroiliitis on radiographs (Best Pract. Res. Clin. Rheumatol. 2010;24:589-604). The other pathway requires HLA-B27 positivity plus two or more SpA features. In patients with peripheral manifestations only, the requirements include peripheral arthritis, enthesitis or dactylitis plus one or more SpA features, including sacroiliitis on imaging.

Dr. Dougados also spoke about recent findings showing that patients with SpA were more likely to have distinct noninflammatory spinal MRI lesions (known as Fatty Romanus lesions) than were patients with degenerative arthritis or spinal malignancy (Ann. Rheum. Dis. 2010; 69:891-94).

"As MRI is becoming more important, rheumatologists should be trained to interpret MRIs," he said. "You don’t need to be a specialist in radiology."

Dr. Dougados has received grants for research projects and/or honorarium fees for participation at advisory boards/symposiums from Abbott, Bristol-Myers Squibb, Merck, Pfizer, Sanofi, and UCB.

NEW YORK – When magnetic resonance imaging is used instead of plain x-rays in patients with early inflammatory back pain, the diagnostic accuracy for spondyloarthritis jumps from 25% to 70%, according to Dr. Maxime Dougados, who spoke at a rheumatology meeting sponsored by New York University.

"Seventy-five percent of the time, you cannot make the diagnosis with plain x-rays," said Dr. Dougados, professor of rheumatology at Paris-Descartes University/Cochin Hospital in Paris and the president-elect of EULAR. He presented the Ira Goldstein Memorial Lecture at the meeting, focusing on spondyloarthritis (SpA).

Dr. Dougados presented the as yet unpublished results from the DESIR cohort, a large French national multicenter database of long-term follow-up of 708 patients presenting with early inflammatory back pain that was initiated by the French Society of Rheumatology. Patients were recruited between December 2007 and April 2010 if they had inflammatory back pain lasting more than 3 months and less than 3 years. The group will be followed for 10 years in the ongoing study. At baseline, the mean age was 35 years, 54% were female and 57% were HLA-B27 positive (Joint Bone Spine 2011 March 30 [doi: 10.1016/j.jbspin.2011.01.013]).

Looking at radiological sacroiliac changes, the diagnosis was "obvious" for 25.6% of the cohort, "doubtful" for 21.3%, and "normal" for 53.1%. "These results indicate that at the first clinical visit, the interview is very important to pick up other clinical symptoms," said Dr. Dougados. In fact, about 80% were found to have non-axial clinical manifestations, including articular peripheral involvement, enthesopathy, dactylitis, anterior chest wall pain, uveitis, or psoriasis.

Using MRI, 70% of the cohort were determined to have "obvious" sacroiliitis, about 20% had a "doubtful" diagnosis and about 10% were thought to be "normal."

"These results indicate that you can detect early abnormalities of the sacroiliac joint on MRI even if x-rays are normal," he said.

According to Dr. Dougados, these imaging findings fit well with recent results from the DECLIC study, in which 163 rheumatologists were asked to diagnose 472 patients with early inflammatory back pain, including 161 patients with spondyloarthritis, according to four different sets of criteria. The specificity of the modified New York criteria, which relies on radiographic signs of sacroiliitis (unilateral grade III or bilateral grade II), fell well below that of the modified Amor criteria, the modified ESSG (European Spondyloarthropathy Study Group) criteria, and the ASAS (Assessment of Spondyloarthritis International Society) criteria. The latter three criteria include the option of diagnosing sacroiliitis with MRI.

In the new classification criteria from the ASAS, separate criteria are listed for patients with axial SpA with and without peripheral manifestations and patients with peripheral manifestations only. For axial SpA, one diagnostic pathway requires sacroiliitis on imaging plus one or more SpA feature. Sacroiliitis on MRI is given as much weight as is sacroiliitis on radiographs (Best Pract. Res. Clin. Rheumatol. 2010;24:589-604). The other pathway requires HLA-B27 positivity plus two or more SpA features. In patients with peripheral manifestations only, the requirements include peripheral arthritis, enthesitis or dactylitis plus one or more SpA features, including sacroiliitis on imaging.

Dr. Dougados also spoke about recent findings showing that patients with SpA were more likely to have distinct noninflammatory spinal MRI lesions (known as Fatty Romanus lesions) than were patients with degenerative arthritis or spinal malignancy (Ann. Rheum. Dis. 2010; 69:891-94).

"As MRI is becoming more important, rheumatologists should be trained to interpret MRIs," he said. "You don’t need to be a specialist in radiology."

Dr. Dougados has received grants for research projects and/or honorarium fees for participation at advisory boards/symposiums from Abbott, Bristol-Myers Squibb, Merck, Pfizer, Sanofi, and UCB.

NEW YORK – When magnetic resonance imaging is used instead of plain x-rays in patients with early inflammatory back pain, the diagnostic accuracy for spondyloarthritis jumps from 25% to 70%, according to Dr. Maxime Dougados, who spoke at a rheumatology meeting sponsored by New York University.

"Seventy-five percent of the time, you cannot make the diagnosis with plain x-rays," said Dr. Dougados, professor of rheumatology at Paris-Descartes University/Cochin Hospital in Paris and the president-elect of EULAR. He presented the Ira Goldstein Memorial Lecture at the meeting, focusing on spondyloarthritis (SpA).

Dr. Dougados presented the as yet unpublished results from the DESIR cohort, a large French national multicenter database of long-term follow-up of 708 patients presenting with early inflammatory back pain that was initiated by the French Society of Rheumatology. Patients were recruited between December 2007 and April 2010 if they had inflammatory back pain lasting more than 3 months and less than 3 years. The group will be followed for 10 years in the ongoing study. At baseline, the mean age was 35 years, 54% were female and 57% were HLA-B27 positive (Joint Bone Spine 2011 March 30 [doi: 10.1016/j.jbspin.2011.01.013]).

Looking at radiological sacroiliac changes, the diagnosis was "obvious" for 25.6% of the cohort, "doubtful" for 21.3%, and "normal" for 53.1%. "These results indicate that at the first clinical visit, the interview is very important to pick up other clinical symptoms," said Dr. Dougados. In fact, about 80% were found to have non-axial clinical manifestations, including articular peripheral involvement, enthesopathy, dactylitis, anterior chest wall pain, uveitis, or psoriasis.

Using MRI, 70% of the cohort were determined to have "obvious" sacroiliitis, about 20% had a "doubtful" diagnosis and about 10% were thought to be "normal."

"These results indicate that you can detect early abnormalities of the sacroiliac joint on MRI even if x-rays are normal," he said.

According to Dr. Dougados, these imaging findings fit well with recent results from the DECLIC study, in which 163 rheumatologists were asked to diagnose 472 patients with early inflammatory back pain, including 161 patients with spondyloarthritis, according to four different sets of criteria. The specificity of the modified New York criteria, which relies on radiographic signs of sacroiliitis (unilateral grade III or bilateral grade II), fell well below that of the modified Amor criteria, the modified ESSG (European Spondyloarthropathy Study Group) criteria, and the ASAS (Assessment of Spondyloarthritis International Society) criteria. The latter three criteria include the option of diagnosing sacroiliitis with MRI.

In the new classification criteria from the ASAS, separate criteria are listed for patients with axial SpA with and without peripheral manifestations and patients with peripheral manifestations only. For axial SpA, one diagnostic pathway requires sacroiliitis on imaging plus one or more SpA feature. Sacroiliitis on MRI is given as much weight as is sacroiliitis on radiographs (Best Pract. Res. Clin. Rheumatol. 2010;24:589-604). The other pathway requires HLA-B27 positivity plus two or more SpA features. In patients with peripheral manifestations only, the requirements include peripheral arthritis, enthesitis or dactylitis plus one or more SpA features, including sacroiliitis on imaging.

Dr. Dougados also spoke about recent findings showing that patients with SpA were more likely to have distinct noninflammatory spinal MRI lesions (known as Fatty Romanus lesions) than were patients with degenerative arthritis or spinal malignancy (Ann. Rheum. Dis. 2010; 69:891-94).

"As MRI is becoming more important, rheumatologists should be trained to interpret MRIs," he said. "You don’t need to be a specialist in radiology."

Dr. Dougados has received grants for research projects and/or honorarium fees for participation at advisory boards/symposiums from Abbott, Bristol-Myers Squibb, Merck, Pfizer, Sanofi, and UCB.

NEW YORK – When magnetic resonance imaging is used instead of plain x-rays in patients with early inflammatory back pain, the diagnostic accuracy for spondyloarthritis jumps from 25% to 70%, according to Dr. Maxime Dougados, who spoke at a rheumatology meeting sponsored by New York University.

"Seventy-five percent of the time, you cannot make the diagnosis with plain x-rays," said Dr. Dougados, professor of rheumatology at Paris-Descartes University/Cochin Hospital in Paris and the president-elect of EULAR. He presented the Ira Goldstein Memorial Lecture at the meeting, focusing on spondyloarthritis (SpA).

Dr. Dougados presented the as yet unpublished results from the DESIR cohort, a large French national multicenter database of long-term follow-up of 708 patients presenting with early inflammatory back pain that was initiated by the French Society of Rheumatology. Patients were recruited between December 2007 and April 2010 if they had inflammatory back pain lasting more than 3 months and less than 3 years. The group will be followed for 10 years in the ongoing study. At baseline, the mean age was 35 years, 54% were female and 57% were HLA-B27 positive (Joint Bone Spine 2011 March 30 [doi: 10.1016/j.jbspin.2011.01.013]).

Looking at radiological sacroiliac changes, the diagnosis was "obvious" for 25.6% of the cohort, "doubtful" for 21.3%, and "normal" for 53.1%. "These results indicate that at the first clinical visit, the interview is very important to pick up other clinical symptoms," said Dr. Dougados. In fact, about 80% were found to have non-axial clinical manifestations, including articular peripheral involvement, enthesopathy, dactylitis, anterior chest wall pain, uveitis, or psoriasis.

Using MRI, 70% of the cohort were determined to have "obvious" sacroiliitis, about 20% had a "doubtful" diagnosis and about 10% were thought to be "normal."

"These results indicate that you can detect early abnormalities of the sacroiliac joint on MRI even if x-rays are normal," he said.

According to Dr. Dougados, these imaging findings fit well with recent results from the DECLIC study, in which 163 rheumatologists were asked to diagnose 472 patients with early inflammatory back pain, including 161 patients with spondyloarthritis, according to four different sets of criteria. The specificity of the modified New York criteria, which relies on radiographic signs of sacroiliitis (unilateral grade III or bilateral grade II), fell well below that of the modified Amor criteria, the modified ESSG (European Spondyloarthropathy Study Group) criteria, and the ASAS (Assessment of Spondyloarthritis International Society) criteria. The latter three criteria include the option of diagnosing sacroiliitis with MRI.

In the new classification criteria from the ASAS, separate criteria are listed for patients with axial SpA with and without peripheral manifestations and patients with peripheral manifestations only. For axial SpA, one diagnostic pathway requires sacroiliitis on imaging plus one or more SpA feature. Sacroiliitis on MRI is given as much weight as is sacroiliitis on radiographs (Best Pract. Res. Clin. Rheumatol. 2010;24:589-604). The other pathway requires HLA-B27 positivity plus two or more SpA features. In patients with peripheral manifestations only, the requirements include peripheral arthritis, enthesitis or dactylitis plus one or more SpA features, including sacroiliitis on imaging.

Dr. Dougados also spoke about recent findings showing that patients with SpA were more likely to have distinct noninflammatory spinal MRI lesions (known as Fatty Romanus lesions) than were patients with degenerative arthritis or spinal malignancy (Ann. Rheum. Dis. 2010; 69:891-94).

"As MRI is becoming more important, rheumatologists should be trained to interpret MRIs," he said. "You don’t need to be a specialist in radiology."

Dr. Dougados has received grants for research projects and/or honorarium fees for participation at advisory boards/symposiums from Abbott, Bristol-Myers Squibb, Merck, Pfizer, Sanofi, and UCB.

NEW YORK – When magnetic resonance imaging is used instead of plain x-rays in patients with early inflammatory back pain, the diagnostic accuracy for spondyloarthritis jumps from 25% to 70%, according to Dr. Maxime Dougados, who spoke at a rheumatology meeting sponsored by New York University.

"Seventy-five percent of the time, you cannot make the diagnosis with plain x-rays," said Dr. Dougados, professor of rheumatology at Paris-Descartes University/Cochin Hospital in Paris and the president-elect of EULAR. He presented the Ira Goldstein Memorial Lecture at the meeting, focusing on spondyloarthritis (SpA).

Dr. Dougados presented the as yet unpublished results from the DESIR cohort, a large French national multicenter database of long-term follow-up of 708 patients presenting with early inflammatory back pain that was initiated by the French Society of Rheumatology. Patients were recruited between December 2007 and April 2010 if they had inflammatory back pain lasting more than 3 months and less than 3 years. The group will be followed for 10 years in the ongoing study. At baseline, the mean age was 35 years, 54% were female and 57% were HLA-B27 positive (Joint Bone Spine 2011 March 30 [doi: 10.1016/j.jbspin.2011.01.013]).

Looking at radiological sacroiliac changes, the diagnosis was "obvious" for 25.6% of the cohort, "doubtful" for 21.3%, and "normal" for 53.1%. "These results indicate that at the first clinical visit, the interview is very important to pick up other clinical symptoms," said Dr. Dougados. In fact, about 80% were found to have non-axial clinical manifestations, including articular peripheral involvement, enthesopathy, dactylitis, anterior chest wall pain, uveitis, or psoriasis.

Using MRI, 70% of the cohort were determined to have "obvious" sacroiliitis, about 20% had a "doubtful" diagnosis and about 10% were thought to be "normal."

"These results indicate that you can detect early abnormalities of the sacroiliac joint on MRI even if x-rays are normal," he said.

According to Dr. Dougados, these imaging findings fit well with recent results from the DECLIC study, in which 163 rheumatologists were asked to diagnose 472 patients with early inflammatory back pain, including 161 patients with spondyloarthritis, according to four different sets of criteria. The specificity of the modified New York criteria, which relies on radiographic signs of sacroiliitis (unilateral grade III or bilateral grade II), fell well below that of the modified Amor criteria, the modified ESSG (European Spondyloarthropathy Study Group) criteria, and the ASAS (Assessment of Spondyloarthritis International Society) criteria. The latter three criteria include the option of diagnosing sacroiliitis with MRI.

In the new classification criteria from the ASAS, separate criteria are listed for patients with axial SpA with and without peripheral manifestations and patients with peripheral manifestations only. For axial SpA, one diagnostic pathway requires sacroiliitis on imaging plus one or more SpA feature. Sacroiliitis on MRI is given as much weight as is sacroiliitis on radiographs (Best Pract. Res. Clin. Rheumatol. 2010;24:589-604). The other pathway requires HLA-B27 positivity plus two or more SpA features. In patients with peripheral manifestations only, the requirements include peripheral arthritis, enthesitis or dactylitis plus one or more SpA features, including sacroiliitis on imaging.

Dr. Dougados also spoke about recent findings showing that patients with SpA were more likely to have distinct noninflammatory spinal MRI lesions (known as Fatty Romanus lesions) than were patients with degenerative arthritis or spinal malignancy (Ann. Rheum. Dis. 2010; 69:891-94).

"As MRI is becoming more important, rheumatologists should be trained to interpret MRIs," he said. "You don’t need to be a specialist in radiology."

Dr. Dougados has received grants for research projects and/or honorarium fees for participation at advisory boards/symposiums from Abbott, Bristol-Myers Squibb, Merck, Pfizer, Sanofi, and UCB.