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JAK inhibitor therapy promising for refractory dermatomyositis
ORLANDO – The according to Ruth Ann Vleugels, MD, director of the autoimmune skin diseases program at Brigham and Women’s Hospital, Boston.
“We will have patients who essentially fail all of our typical therapies and are still coming to us for help. This is a huge challenge,” said Dr. Vleugels, who, several years ago, started to use tofacitinib to treat these patients. “Similar to my colleagues who use tofacitinib to treat alopecia areata, we often have to push” beyond the dose used to treat rheumatoid arthritis, to 10 mg twice a day, she said at the International Conference on Cutaneous Lupus Erythematosus. Tofacitinib helps counter the overexpression of interferon in DM.
Getting insurance coverage for this off-label indication can be tough, however, but Dr. Vleugels said she’s had success when she tells insurers that tofacitinib will likely reduce the need for IVIg.
It’s safe to keep patients on methotrexate if there are concerns about muscle involvement while their skin is brought under control with tofacitinib. In terms of side effects, “we see increased shingles,” so recommending the shingles vaccine for these patients is a good idea, she added.
It’s also important to counsel DM patients that they are at particular risk for skin reactions with antimalarials, which can be serious, so that, “if there is a drug reaction that develops, it’s noticed right away” and the drug can be stopped, she said. “If you have a patient who has very severe disease, I might skip over an antimalarial altogether,” she commented.
Methotrexate is the next option, especially if there are work ups for cancer or the patients have cancer, but Dr. Femia, director of inpatient dermatology at NYU, said she leans towards mycophenolate if there’s concern about lung involvement.
The next step, if necessary, is IVIg, which she said is “particularly helpful” for recalcitrant skin disease and can help some patients discontinue other immunosuppressives. To counter headache, a common side effect, she will space dosing out over 3 days, instead of the usual 2, and have a bag of saline administered before and after the infusion to keep patients hydrated; this counters the headache-inducing viscosity of IVIg.
Patients often see a result after the first infusion, but if there’s no benefit by the third cycle, “it’s probably time to move on,” she said. “If you have a refractory muscle disease patient and skin isn’t the main issue, rituximab is reasonable to try,” she added, noting that the benefit of tumor necrosis factor blockers, “at best, is very mixed in the DM population. They are very low down in the treatment algorithm.”
Dr. Vleugels and Dr. Femia are both Pfizer investigators.
ORLANDO – The according to Ruth Ann Vleugels, MD, director of the autoimmune skin diseases program at Brigham and Women’s Hospital, Boston.
“We will have patients who essentially fail all of our typical therapies and are still coming to us for help. This is a huge challenge,” said Dr. Vleugels, who, several years ago, started to use tofacitinib to treat these patients. “Similar to my colleagues who use tofacitinib to treat alopecia areata, we often have to push” beyond the dose used to treat rheumatoid arthritis, to 10 mg twice a day, she said at the International Conference on Cutaneous Lupus Erythematosus. Tofacitinib helps counter the overexpression of interferon in DM.
Getting insurance coverage for this off-label indication can be tough, however, but Dr. Vleugels said she’s had success when she tells insurers that tofacitinib will likely reduce the need for IVIg.
It’s safe to keep patients on methotrexate if there are concerns about muscle involvement while their skin is brought under control with tofacitinib. In terms of side effects, “we see increased shingles,” so recommending the shingles vaccine for these patients is a good idea, she added.
It’s also important to counsel DM patients that they are at particular risk for skin reactions with antimalarials, which can be serious, so that, “if there is a drug reaction that develops, it’s noticed right away” and the drug can be stopped, she said. “If you have a patient who has very severe disease, I might skip over an antimalarial altogether,” she commented.
Methotrexate is the next option, especially if there are work ups for cancer or the patients have cancer, but Dr. Femia, director of inpatient dermatology at NYU, said she leans towards mycophenolate if there’s concern about lung involvement.
The next step, if necessary, is IVIg, which she said is “particularly helpful” for recalcitrant skin disease and can help some patients discontinue other immunosuppressives. To counter headache, a common side effect, she will space dosing out over 3 days, instead of the usual 2, and have a bag of saline administered before and after the infusion to keep patients hydrated; this counters the headache-inducing viscosity of IVIg.
Patients often see a result after the first infusion, but if there’s no benefit by the third cycle, “it’s probably time to move on,” she said. “If you have a refractory muscle disease patient and skin isn’t the main issue, rituximab is reasonable to try,” she added, noting that the benefit of tumor necrosis factor blockers, “at best, is very mixed in the DM population. They are very low down in the treatment algorithm.”
Dr. Vleugels and Dr. Femia are both Pfizer investigators.
ORLANDO – The according to Ruth Ann Vleugels, MD, director of the autoimmune skin diseases program at Brigham and Women’s Hospital, Boston.
“We will have patients who essentially fail all of our typical therapies and are still coming to us for help. This is a huge challenge,” said Dr. Vleugels, who, several years ago, started to use tofacitinib to treat these patients. “Similar to my colleagues who use tofacitinib to treat alopecia areata, we often have to push” beyond the dose used to treat rheumatoid arthritis, to 10 mg twice a day, she said at the International Conference on Cutaneous Lupus Erythematosus. Tofacitinib helps counter the overexpression of interferon in DM.
Getting insurance coverage for this off-label indication can be tough, however, but Dr. Vleugels said she’s had success when she tells insurers that tofacitinib will likely reduce the need for IVIg.
It’s safe to keep patients on methotrexate if there are concerns about muscle involvement while their skin is brought under control with tofacitinib. In terms of side effects, “we see increased shingles,” so recommending the shingles vaccine for these patients is a good idea, she added.
It’s also important to counsel DM patients that they are at particular risk for skin reactions with antimalarials, which can be serious, so that, “if there is a drug reaction that develops, it’s noticed right away” and the drug can be stopped, she said. “If you have a patient who has very severe disease, I might skip over an antimalarial altogether,” she commented.
Methotrexate is the next option, especially if there are work ups for cancer or the patients have cancer, but Dr. Femia, director of inpatient dermatology at NYU, said she leans towards mycophenolate if there’s concern about lung involvement.
The next step, if necessary, is IVIg, which she said is “particularly helpful” for recalcitrant skin disease and can help some patients discontinue other immunosuppressives. To counter headache, a common side effect, she will space dosing out over 3 days, instead of the usual 2, and have a bag of saline administered before and after the infusion to keep patients hydrated; this counters the headache-inducing viscosity of IVIg.
Patients often see a result after the first infusion, but if there’s no benefit by the third cycle, “it’s probably time to move on,” she said. “If you have a refractory muscle disease patient and skin isn’t the main issue, rituximab is reasonable to try,” she added, noting that the benefit of tumor necrosis factor blockers, “at best, is very mixed in the DM population. They are very low down in the treatment algorithm.”
Dr. Vleugels and Dr. Femia are both Pfizer investigators.
EXPERT ANALYSIS FROM ICCLE 2018
Cutaneous lupus: Switching antimalarials can delay immunosuppressive therapy
ORLANDO – , according to Anthony Fernandez, MD, PhD, director of medical and inpatient dermatology at the Cleveland Clinic.
A switch to chloroquine, or adding quinacrine, might do the trick, saving at least some patients from having to move on to immunosuppressive therapy, Dr. Fernandez said at the International Conference on Cutaneous Lupus Erythematosus.
“What we are learning from the literature is that we can switch from one antimalarial to another. We need to think about this in our algorithms before reaching for potentially more toxic immunosuppressives,” Dr. Fernandez said.
As for quinacrine, about two-thirds of patients who fail hydroxychloroquine or chloroquine will have a positive response to quinacrine if added (Br J Dermatol. 2017 Jul;177[1]:188-96). “It’s important to remember that we are not adding any ocular toxicity” with quinacrine, he said.
Quinacrine does come with a major concern of its own: the risk of aplastic anemia. However, this seems to occur with doses higher than 100 mg/day, which are no longer recommended; there have been no reports of aplastic anemia in patients on 100 mg/day or less.
Quinacrine “is an underutilized antimalarial. I think a lot of people don’t know about it or know how to get it. It can be compounded into capsules for patients,” and for a reasonable price, at about $20 for a month supply at some pharmacies, Dr. Fernandez said.
Meanwhile, because of the risk of retinal toxicity with hydroxychloroquine, there’s been a shift in recent years from dosing up to 6.5 mg/kg per day of ideal body weight to a ceiling of 5 mg/kg per day of actual body weight (JAMA Ophthalmol. 2014 Dec;132[12]:1453-60), and a ceiling of 2.3 mg/kg per day actual body weight for chloroquine.
The idea was to prevent overdosing in people who are under their ideal body weight, but there have been concerns about the efficacy of the new dosing regimen in other patients. Dr. Fernandez has not seen evidence of this. “We do adhere to the new dosing recommendations” at the Cleveland Clinic, and “personally, I think we are seeing similar efficacy,” he said.
The most important risk factor for retinal toxicity is cumulative dose. The risk seems to be extremely low in the first 5 years, but increases afterwards. Most patients who develop retinal toxicity have taken a cumulative hydroxychloroquine dose of 1,000 g, equal to about 400 mg/d for 7 years. “The longer you are on the medicine, the higher your risk of developing retinal toxicity,” he noted.
Regardless of weight, it’s recommended to limit hydroxychloroquine to 400 mg daily and chloroquine to 250 mg daily, with a baseline ocular exam, and – barring any intervening problems – annual screening after 5 years.
In patients with highly active skin disease at baseline, Dr. Fernandez said he will sometimes start hydroxychloroquine higher than 400 mg initially but will bring patients back down to 400 mg after a few months.
Dr. Fernandez had no relevant disclosures.
ORLANDO – , according to Anthony Fernandez, MD, PhD, director of medical and inpatient dermatology at the Cleveland Clinic.
A switch to chloroquine, or adding quinacrine, might do the trick, saving at least some patients from having to move on to immunosuppressive therapy, Dr. Fernandez said at the International Conference on Cutaneous Lupus Erythematosus.
“What we are learning from the literature is that we can switch from one antimalarial to another. We need to think about this in our algorithms before reaching for potentially more toxic immunosuppressives,” Dr. Fernandez said.
As for quinacrine, about two-thirds of patients who fail hydroxychloroquine or chloroquine will have a positive response to quinacrine if added (Br J Dermatol. 2017 Jul;177[1]:188-96). “It’s important to remember that we are not adding any ocular toxicity” with quinacrine, he said.
Quinacrine does come with a major concern of its own: the risk of aplastic anemia. However, this seems to occur with doses higher than 100 mg/day, which are no longer recommended; there have been no reports of aplastic anemia in patients on 100 mg/day or less.
Quinacrine “is an underutilized antimalarial. I think a lot of people don’t know about it or know how to get it. It can be compounded into capsules for patients,” and for a reasonable price, at about $20 for a month supply at some pharmacies, Dr. Fernandez said.
Meanwhile, because of the risk of retinal toxicity with hydroxychloroquine, there’s been a shift in recent years from dosing up to 6.5 mg/kg per day of ideal body weight to a ceiling of 5 mg/kg per day of actual body weight (JAMA Ophthalmol. 2014 Dec;132[12]:1453-60), and a ceiling of 2.3 mg/kg per day actual body weight for chloroquine.
The idea was to prevent overdosing in people who are under their ideal body weight, but there have been concerns about the efficacy of the new dosing regimen in other patients. Dr. Fernandez has not seen evidence of this. “We do adhere to the new dosing recommendations” at the Cleveland Clinic, and “personally, I think we are seeing similar efficacy,” he said.
The most important risk factor for retinal toxicity is cumulative dose. The risk seems to be extremely low in the first 5 years, but increases afterwards. Most patients who develop retinal toxicity have taken a cumulative hydroxychloroquine dose of 1,000 g, equal to about 400 mg/d for 7 years. “The longer you are on the medicine, the higher your risk of developing retinal toxicity,” he noted.
Regardless of weight, it’s recommended to limit hydroxychloroquine to 400 mg daily and chloroquine to 250 mg daily, with a baseline ocular exam, and – barring any intervening problems – annual screening after 5 years.
In patients with highly active skin disease at baseline, Dr. Fernandez said he will sometimes start hydroxychloroquine higher than 400 mg initially but will bring patients back down to 400 mg after a few months.
Dr. Fernandez had no relevant disclosures.
ORLANDO – , according to Anthony Fernandez, MD, PhD, director of medical and inpatient dermatology at the Cleveland Clinic.
A switch to chloroquine, or adding quinacrine, might do the trick, saving at least some patients from having to move on to immunosuppressive therapy, Dr. Fernandez said at the International Conference on Cutaneous Lupus Erythematosus.
“What we are learning from the literature is that we can switch from one antimalarial to another. We need to think about this in our algorithms before reaching for potentially more toxic immunosuppressives,” Dr. Fernandez said.
As for quinacrine, about two-thirds of patients who fail hydroxychloroquine or chloroquine will have a positive response to quinacrine if added (Br J Dermatol. 2017 Jul;177[1]:188-96). “It’s important to remember that we are not adding any ocular toxicity” with quinacrine, he said.
Quinacrine does come with a major concern of its own: the risk of aplastic anemia. However, this seems to occur with doses higher than 100 mg/day, which are no longer recommended; there have been no reports of aplastic anemia in patients on 100 mg/day or less.
Quinacrine “is an underutilized antimalarial. I think a lot of people don’t know about it or know how to get it. It can be compounded into capsules for patients,” and for a reasonable price, at about $20 for a month supply at some pharmacies, Dr. Fernandez said.
Meanwhile, because of the risk of retinal toxicity with hydroxychloroquine, there’s been a shift in recent years from dosing up to 6.5 mg/kg per day of ideal body weight to a ceiling of 5 mg/kg per day of actual body weight (JAMA Ophthalmol. 2014 Dec;132[12]:1453-60), and a ceiling of 2.3 mg/kg per day actual body weight for chloroquine.
The idea was to prevent overdosing in people who are under their ideal body weight, but there have been concerns about the efficacy of the new dosing regimen in other patients. Dr. Fernandez has not seen evidence of this. “We do adhere to the new dosing recommendations” at the Cleveland Clinic, and “personally, I think we are seeing similar efficacy,” he said.
The most important risk factor for retinal toxicity is cumulative dose. The risk seems to be extremely low in the first 5 years, but increases afterwards. Most patients who develop retinal toxicity have taken a cumulative hydroxychloroquine dose of 1,000 g, equal to about 400 mg/d for 7 years. “The longer you are on the medicine, the higher your risk of developing retinal toxicity,” he noted.
Regardless of weight, it’s recommended to limit hydroxychloroquine to 400 mg daily and chloroquine to 250 mg daily, with a baseline ocular exam, and – barring any intervening problems – annual screening after 5 years.
In patients with highly active skin disease at baseline, Dr. Fernandez said he will sometimes start hydroxychloroquine higher than 400 mg initially but will bring patients back down to 400 mg after a few months.
Dr. Fernandez had no relevant disclosures.
EXPERT ANALYSIS FROM ICCLE 2018
Hydroxychloroquine throws off Quantiferon-TB Gold results, study finds
ORLANDO – according to investigators from the University of Pennsylvania, Philadelphia.
Among 46 patients with lupus, dermatomyositis, or blistering diseases who had been on hydroxychloroquine within a year of testing, QuantiFERON-TB Gold (QFT-G) – the go-to TB test in many places – yielded indeterminate results in 37%. Meanwhile, just 9.6% of tests were indeterminate among 73 patients with those diseases who had not been on hydroxychloroquine (P less than .001). The findings could not be explained by concomitant use of prednisone and other immunosuppressives; there were no statistically significant differences between the groups. “This was shocking to us. We need to come up with a better screening test in this patient population,” said lead investigator Rebecca Gaffney, a research fellow at the University of Pennsylvania, and a medical student at Robert Wood Johnson Medical School, New Brunswick, NJ.*
It’s widely known that immunosuppressives interfere with QFT-G results, but antimalarials are considered immunomodulators, not immunosuppressives. The new study is probably the first to investigate the issue. The team is now pitting QFT-G against another TB blood test, the T-SPOT, in 100 patients to see if it’s a better option, in a trial that they expect to complete in 2018.
The investigators have a hunch that the T-SPOT might be better because, while QFT-G measures interferon-gamma concentrations in response to TB antigens, the T-SPOT “counts cells first to make sure you have a standard amount of cells, then looks at how many cells are releasing interferon-gamma,” Ms. Gaffney said, adding that “it seems like a more sensitive test,” especially for lymphocytopenic autoimmune patients. “We are really excited to see if there’s a better test for our patients, given all the clinical trials we do. We want to see what’s best, so there’s no barrier to receiving therapy.”
Subjects were around 50 years old on average, and the majority were women. Most were white, and about 20% were black.
There was no industry funding for the work, and Ms. Gaffney reported no disclosures.
*This article was updated on June 13. 2018.
ORLANDO – according to investigators from the University of Pennsylvania, Philadelphia.
Among 46 patients with lupus, dermatomyositis, or blistering diseases who had been on hydroxychloroquine within a year of testing, QuantiFERON-TB Gold (QFT-G) – the go-to TB test in many places – yielded indeterminate results in 37%. Meanwhile, just 9.6% of tests were indeterminate among 73 patients with those diseases who had not been on hydroxychloroquine (P less than .001). The findings could not be explained by concomitant use of prednisone and other immunosuppressives; there were no statistically significant differences between the groups. “This was shocking to us. We need to come up with a better screening test in this patient population,” said lead investigator Rebecca Gaffney, a research fellow at the University of Pennsylvania, and a medical student at Robert Wood Johnson Medical School, New Brunswick, NJ.*
It’s widely known that immunosuppressives interfere with QFT-G results, but antimalarials are considered immunomodulators, not immunosuppressives. The new study is probably the first to investigate the issue. The team is now pitting QFT-G against another TB blood test, the T-SPOT, in 100 patients to see if it’s a better option, in a trial that they expect to complete in 2018.
The investigators have a hunch that the T-SPOT might be better because, while QFT-G measures interferon-gamma concentrations in response to TB antigens, the T-SPOT “counts cells first to make sure you have a standard amount of cells, then looks at how many cells are releasing interferon-gamma,” Ms. Gaffney said, adding that “it seems like a more sensitive test,” especially for lymphocytopenic autoimmune patients. “We are really excited to see if there’s a better test for our patients, given all the clinical trials we do. We want to see what’s best, so there’s no barrier to receiving therapy.”
Subjects were around 50 years old on average, and the majority were women. Most were white, and about 20% were black.
There was no industry funding for the work, and Ms. Gaffney reported no disclosures.
*This article was updated on June 13. 2018.
ORLANDO – according to investigators from the University of Pennsylvania, Philadelphia.
Among 46 patients with lupus, dermatomyositis, or blistering diseases who had been on hydroxychloroquine within a year of testing, QuantiFERON-TB Gold (QFT-G) – the go-to TB test in many places – yielded indeterminate results in 37%. Meanwhile, just 9.6% of tests were indeterminate among 73 patients with those diseases who had not been on hydroxychloroquine (P less than .001). The findings could not be explained by concomitant use of prednisone and other immunosuppressives; there were no statistically significant differences between the groups. “This was shocking to us. We need to come up with a better screening test in this patient population,” said lead investigator Rebecca Gaffney, a research fellow at the University of Pennsylvania, and a medical student at Robert Wood Johnson Medical School, New Brunswick, NJ.*
It’s widely known that immunosuppressives interfere with QFT-G results, but antimalarials are considered immunomodulators, not immunosuppressives. The new study is probably the first to investigate the issue. The team is now pitting QFT-G against another TB blood test, the T-SPOT, in 100 patients to see if it’s a better option, in a trial that they expect to complete in 2018.
The investigators have a hunch that the T-SPOT might be better because, while QFT-G measures interferon-gamma concentrations in response to TB antigens, the T-SPOT “counts cells first to make sure you have a standard amount of cells, then looks at how many cells are releasing interferon-gamma,” Ms. Gaffney said, adding that “it seems like a more sensitive test,” especially for lymphocytopenic autoimmune patients. “We are really excited to see if there’s a better test for our patients, given all the clinical trials we do. We want to see what’s best, so there’s no barrier to receiving therapy.”
Subjects were around 50 years old on average, and the majority were women. Most were white, and about 20% were black.
There was no industry funding for the work, and Ms. Gaffney reported no disclosures.
*This article was updated on June 13. 2018.
REPORTING FROM ICCLE 2018
Median time to progression from discoid to systemic lupus may be less than a year
ORLANDO – Progression from cutaneous to systemic lupus happens far more quickly than is commonly reported in the literature, according to an investigation from Brigham and Women’s Hospital, Boston.
Most studies have emphasized mean time to progression, which is generally said to be about 8 years. The problem is that the range is broad, anywhere from a few months to 30 or more years, so “the outliers influence the mean, and therefore, may not provide a fully accurate representation of the interval in which [most] patients progress” from discoid lupus erythematosus (DLE) to systemic lupus erythematosus (SLE), lead investigator Scott Elman, MD, said at the International Conference on Cutaneous Lupus Erythematosus.
He and his team thought it would be more useful to instead look at median time to progression, the point at which half of patients develop systemic disease. They found that , with the first quartile progressing by 303 days. The mean time to progression, meanwhile, was over 4 years, because of outliers who progressed at anywhere from 27 days to 30 years.
“The median progression of DLE to SLE occurs much sooner than previously reported” with mean progression times, said Dr. Elman, an internal medicine and dermatology resident at Brigham and Women’s. “We believe the use of median [time] is [more] clinically useful for both providers and patients in their understanding of what a diagnosis of DLE means for the risk of developing systemic disease.
“As clinicians seeing DLE patients, the primary question we are asking ourselves is how frequently we should be monitoring for signs and symptoms of systemic lupus,” he said. Relying on mean time instead of median time might give a false sense of security – and even delay checking for arthritis, nephritis, and other manifestations. There’s no current standard of practice about when monitoring should occur, but “I certainly think that closer monitoring of new patients with a diagnosis of DLE, especially in their first couple of years,” is a good idea, he noted, adding that for most patients, “it seems that if it’s going to happen, it’s going to happen sooner” rather than later. At Brigham and Women’s, newly diagnosed patients are generally brought back every 3-6 months for lab tests and a thorough review of symptoms, Dr. Elman said.
The next step is to identify risk factors for early progression. The study did not find any significant differences in lab values, medication exposures, or disease manifestations between early and late progressors, perhaps because of the small sample size. The work continues.
There was no external funding for the work. Dr. Elman had no disclosures.
ORLANDO – Progression from cutaneous to systemic lupus happens far more quickly than is commonly reported in the literature, according to an investigation from Brigham and Women’s Hospital, Boston.
Most studies have emphasized mean time to progression, which is generally said to be about 8 years. The problem is that the range is broad, anywhere from a few months to 30 or more years, so “the outliers influence the mean, and therefore, may not provide a fully accurate representation of the interval in which [most] patients progress” from discoid lupus erythematosus (DLE) to systemic lupus erythematosus (SLE), lead investigator Scott Elman, MD, said at the International Conference on Cutaneous Lupus Erythematosus.
He and his team thought it would be more useful to instead look at median time to progression, the point at which half of patients develop systemic disease. They found that , with the first quartile progressing by 303 days. The mean time to progression, meanwhile, was over 4 years, because of outliers who progressed at anywhere from 27 days to 30 years.
“The median progression of DLE to SLE occurs much sooner than previously reported” with mean progression times, said Dr. Elman, an internal medicine and dermatology resident at Brigham and Women’s. “We believe the use of median [time] is [more] clinically useful for both providers and patients in their understanding of what a diagnosis of DLE means for the risk of developing systemic disease.
“As clinicians seeing DLE patients, the primary question we are asking ourselves is how frequently we should be monitoring for signs and symptoms of systemic lupus,” he said. Relying on mean time instead of median time might give a false sense of security – and even delay checking for arthritis, nephritis, and other manifestations. There’s no current standard of practice about when monitoring should occur, but “I certainly think that closer monitoring of new patients with a diagnosis of DLE, especially in their first couple of years,” is a good idea, he noted, adding that for most patients, “it seems that if it’s going to happen, it’s going to happen sooner” rather than later. At Brigham and Women’s, newly diagnosed patients are generally brought back every 3-6 months for lab tests and a thorough review of symptoms, Dr. Elman said.
The next step is to identify risk factors for early progression. The study did not find any significant differences in lab values, medication exposures, or disease manifestations between early and late progressors, perhaps because of the small sample size. The work continues.
There was no external funding for the work. Dr. Elman had no disclosures.
ORLANDO – Progression from cutaneous to systemic lupus happens far more quickly than is commonly reported in the literature, according to an investigation from Brigham and Women’s Hospital, Boston.
Most studies have emphasized mean time to progression, which is generally said to be about 8 years. The problem is that the range is broad, anywhere from a few months to 30 or more years, so “the outliers influence the mean, and therefore, may not provide a fully accurate representation of the interval in which [most] patients progress” from discoid lupus erythematosus (DLE) to systemic lupus erythematosus (SLE), lead investigator Scott Elman, MD, said at the International Conference on Cutaneous Lupus Erythematosus.
He and his team thought it would be more useful to instead look at median time to progression, the point at which half of patients develop systemic disease. They found that , with the first quartile progressing by 303 days. The mean time to progression, meanwhile, was over 4 years, because of outliers who progressed at anywhere from 27 days to 30 years.
“The median progression of DLE to SLE occurs much sooner than previously reported” with mean progression times, said Dr. Elman, an internal medicine and dermatology resident at Brigham and Women’s. “We believe the use of median [time] is [more] clinically useful for both providers and patients in their understanding of what a diagnosis of DLE means for the risk of developing systemic disease.
“As clinicians seeing DLE patients, the primary question we are asking ourselves is how frequently we should be monitoring for signs and symptoms of systemic lupus,” he said. Relying on mean time instead of median time might give a false sense of security – and even delay checking for arthritis, nephritis, and other manifestations. There’s no current standard of practice about when monitoring should occur, but “I certainly think that closer monitoring of new patients with a diagnosis of DLE, especially in their first couple of years,” is a good idea, he noted, adding that for most patients, “it seems that if it’s going to happen, it’s going to happen sooner” rather than later. At Brigham and Women’s, newly diagnosed patients are generally brought back every 3-6 months for lab tests and a thorough review of symptoms, Dr. Elman said.
The next step is to identify risk factors for early progression. The study did not find any significant differences in lab values, medication exposures, or disease manifestations between early and late progressors, perhaps because of the small sample size. The work continues.
There was no external funding for the work. Dr. Elman had no disclosures.
REPORTING FROM ICCLE 2018
Key clinical point: Progression from cutaneous to systemic lupus happens far more quickly than is commonly reported in the literature.
Major finding: The median time to progression was 398 days, among a group of patients diagnosed with discoid lupus erythematosus.
Study details: A review of 32 patients who converted to systemic disease.
Disclosures: There was no external funding for the work, and the lead investigator had no disclosures.
Don’t trust interface dermatitis to diagnose dermatomyositis
ORLANDO – (DM), according to David Fiorentino, MD, PhD, professor of dermatology, rheumatology, and immunology at Stanford (Calif.) University.
The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
Interface dermatitis on skin biopsy is “felt to be almost required by many people to make the diagnosis,” but he and his associates found that it was not present in about a quarter of a cohort of patients with DM. “We don’t want a clinician” to rule out the diagnosis based on its absence on a biopsy, “when its actually quite possible that the patient could have disease,” Dr. Fiorentino said at the International Conference on Cutaneous Lupus Erythematosus.
In general, skin biopsies in DM are tricky. “All of us take them, but we don’t really know how to interpret the information that comes back ... because we don’t really know how often many of [the associated] findings are seen” in DM patients, he noted.
One of the main concerns is to rule out lupus, but interface dermatitis is found in many of its cutaneous forms, as well as in graft-versus-host disease and other diseases.
So what’s a clinician to do? Fortunately, direct immunofluorescence can help. A positive lupus band test helps rule out DM, and the membrane attack complex helps rule it in, both with a good degree of certainty. In a video interview, Dr. Fiorentino explained these tests and how to use them.
Dr. Fiorentino had no relevant disclosures.
aotto@mdedge.com
ORLANDO – (DM), according to David Fiorentino, MD, PhD, professor of dermatology, rheumatology, and immunology at Stanford (Calif.) University.
The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
Interface dermatitis on skin biopsy is “felt to be almost required by many people to make the diagnosis,” but he and his associates found that it was not present in about a quarter of a cohort of patients with DM. “We don’t want a clinician” to rule out the diagnosis based on its absence on a biopsy, “when its actually quite possible that the patient could have disease,” Dr. Fiorentino said at the International Conference on Cutaneous Lupus Erythematosus.
In general, skin biopsies in DM are tricky. “All of us take them, but we don’t really know how to interpret the information that comes back ... because we don’t really know how often many of [the associated] findings are seen” in DM patients, he noted.
One of the main concerns is to rule out lupus, but interface dermatitis is found in many of its cutaneous forms, as well as in graft-versus-host disease and other diseases.
So what’s a clinician to do? Fortunately, direct immunofluorescence can help. A positive lupus band test helps rule out DM, and the membrane attack complex helps rule it in, both with a good degree of certainty. In a video interview, Dr. Fiorentino explained these tests and how to use them.
Dr. Fiorentino had no relevant disclosures.
aotto@mdedge.com
ORLANDO – (DM), according to David Fiorentino, MD, PhD, professor of dermatology, rheumatology, and immunology at Stanford (Calif.) University.
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Interface dermatitis on skin biopsy is “felt to be almost required by many people to make the diagnosis,” but he and his associates found that it was not present in about a quarter of a cohort of patients with DM. “We don’t want a clinician” to rule out the diagnosis based on its absence on a biopsy, “when its actually quite possible that the patient could have disease,” Dr. Fiorentino said at the International Conference on Cutaneous Lupus Erythematosus.
In general, skin biopsies in DM are tricky. “All of us take them, but we don’t really know how to interpret the information that comes back ... because we don’t really know how often many of [the associated] findings are seen” in DM patients, he noted.
One of the main concerns is to rule out lupus, but interface dermatitis is found in many of its cutaneous forms, as well as in graft-versus-host disease and other diseases.
So what’s a clinician to do? Fortunately, direct immunofluorescence can help. A positive lupus band test helps rule out DM, and the membrane attack complex helps rule it in, both with a good degree of certainty. In a video interview, Dr. Fiorentino explained these tests and how to use them.
Dr. Fiorentino had no relevant disclosures.
aotto@mdedge.com
REPORTING FROM ICCLE 2018
