The Journal of Family Practice

The goal of this activity is to update primary care practitioners (PCPs) on risk factors and trends in hepatocellular carcinoma (HCC) development, as well as guideline recommendations and best practices for collaborating with specialists in HCC surveillance.

Click here to access this content now 

The goal of this activity is to update primary care practitioners (PCPs) on risk factors and trends in hepatocellular carcinoma (HCC) development, as well as guideline recommendations and best practices for collaborating with specialists in HCC surveillance.

Click here to access this content now 

The goal of this activity is to update primary care practitioners (PCPs) on risk factors and trends in hepatocellular carcinoma (HCC) development, as well as guideline recommendations and best practices for collaborating with specialists in HCC surveillance.

Click here to access this content now 

Scaly plaques on sun-exposed skin with hyperpigmentation and dyspigmentation are classic signs of cutaneous lupus erythematosus (CLE). (The dyspigmentation seen in this case signaled that she likely had chronic cutaneous lupus erythematosus [CCLE]—a subtype of CLE.) At the patient’s follow-up primary care visit, her antinuclear antibodies titer was 1:1280 (≥ 1:160 is considered a positive test) and her 24-hour urine protein was 1188 mg (normal levels in adults, < 150 mg/d). In light of the patient’s joint pain, lab findings, and skin manifestations, she was also given a diagnosis of systemic lupus erythematosus (SLE).

Lupus erythematosus has an increased prevalence in women and typically occurs between the ages of 20 to 50 years.¹ The incidence and prevalence of this condition is also greater in Black patients. CLE can either occur with SLE or independently. Patients with CLE should be monitored for the development of SLE. A diagnosis of CLE is based mainly on clinical features; biopsy is only indicated if there is a high degree of uncertainty.

Patients with CLE may suffer from a lower quality of life compared to patients with other dermatologic conditions due to the often disfiguring and disabling nature of the condition.^1,2 Additionally, Black patients have an even higher chance of developing depressive symptoms associated with CCLE.²

Therapeutic management for CLE involves photoprotection by wearing sun-protective clothing, sunscreen, and limiting sun exposure.¹ Initial treatment includes topical or intralesional corticosteroids, or topical calcineurin inhibitors. Systemic therapy is similar to that used for SLE. Oral glucocorticoids, and antimalarial agents are considered first-line systemic therapy.¹ Second-line treatment includes methotrexate, mycophenolate mofetil, systemic retinoids, and azathioprine. Other immunosuppressive agents that are less commonly used include clofazimine, cyclophosphamide, and rituximab.¹

The patient was treated sequentially with trials of oral azathioprine 50 mg bid, then prednisone 10 mg once daily, and then hydroxychloroquine 400 mg daily, without significant change in her condition. Additionally, topical steroids did not improve the patient’s symptoms. She was subsequently started on rituximab 1000 mg intravenously with a second dose repeated 2 weeks later, and another treatment 6 months after that. One year after her visit to the ED, the patient was experiencing marked improvement in her lesions.

‌

Photo courtesy of Christy Nwankwo BA. Text courtesy of Christy Nwankwo, BA, University of Missouri-Kansas City School of Medicine and Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque

Scaly plaques on sun-exposed skin with hyperpigmentation and dyspigmentation are classic signs of cutaneous lupus erythematosus (CLE). (The dyspigmentation seen in this case signaled that she likely had chronic cutaneous lupus erythematosus [CCLE]—a subtype of CLE.) At the patient’s follow-up primary care visit, her antinuclear antibodies titer was 1:1280 (≥ 1:160 is considered a positive test) and her 24-hour urine protein was 1188 mg (normal levels in adults, < 150 mg/d). In light of the patient’s joint pain, lab findings, and skin manifestations, she was also given a diagnosis of systemic lupus erythematosus (SLE).

Lupus erythematosus has an increased prevalence in women and typically occurs between the ages of 20 to 50 years.¹ The incidence and prevalence of this condition is also greater in Black patients. CLE can either occur with SLE or independently. Patients with CLE should be monitored for the development of SLE. A diagnosis of CLE is based mainly on clinical features; biopsy is only indicated if there is a high degree of uncertainty.

Patients with CLE may suffer from a lower quality of life compared to patients with other dermatologic conditions due to the often disfiguring and disabling nature of the condition.^1,2 Additionally, Black patients have an even higher chance of developing depressive symptoms associated with CCLE.²

Therapeutic management for CLE involves photoprotection by wearing sun-protective clothing, sunscreen, and limiting sun exposure.¹ Initial treatment includes topical or intralesional corticosteroids, or topical calcineurin inhibitors. Systemic therapy is similar to that used for SLE. Oral glucocorticoids, and antimalarial agents are considered first-line systemic therapy.¹ Second-line treatment includes methotrexate, mycophenolate mofetil, systemic retinoids, and azathioprine. Other immunosuppressive agents that are less commonly used include clofazimine, cyclophosphamide, and rituximab.¹

The patient was treated sequentially with trials of oral azathioprine 50 mg bid, then prednisone 10 mg once daily, and then hydroxychloroquine 400 mg daily, without significant change in her condition. Additionally, topical steroids did not improve the patient’s symptoms. She was subsequently started on rituximab 1000 mg intravenously with a second dose repeated 2 weeks later, and another treatment 6 months after that. One year after her visit to the ED, the patient was experiencing marked improvement in her lesions.

‌

Photo courtesy of Christy Nwankwo BA. Text courtesy of Christy Nwankwo, BA, University of Missouri-Kansas City School of Medicine and Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque

Scaly plaques on sun-exposed skin with hyperpigmentation and dyspigmentation are classic signs of cutaneous lupus erythematosus (CLE). (The dyspigmentation seen in this case signaled that she likely had chronic cutaneous lupus erythematosus [CCLE]—a subtype of CLE.) At the patient’s follow-up primary care visit, her antinuclear antibodies titer was 1:1280 (≥ 1:160 is considered a positive test) and her 24-hour urine protein was 1188 mg (normal levels in adults, < 150 mg/d). In light of the patient’s joint pain, lab findings, and skin manifestations, she was also given a diagnosis of systemic lupus erythematosus (SLE).

Lupus erythematosus has an increased prevalence in women and typically occurs between the ages of 20 to 50 years.¹ The incidence and prevalence of this condition is also greater in Black patients. CLE can either occur with SLE or independently. Patients with CLE should be monitored for the development of SLE. A diagnosis of CLE is based mainly on clinical features; biopsy is only indicated if there is a high degree of uncertainty.

Patients with CLE may suffer from a lower quality of life compared to patients with other dermatologic conditions due to the often disfiguring and disabling nature of the condition.^1,2 Additionally, Black patients have an even higher chance of developing depressive symptoms associated with CCLE.²

Therapeutic management for CLE involves photoprotection by wearing sun-protective clothing, sunscreen, and limiting sun exposure.¹ Initial treatment includes topical or intralesional corticosteroids, or topical calcineurin inhibitors. Systemic therapy is similar to that used for SLE. Oral glucocorticoids, and antimalarial agents are considered first-line systemic therapy.¹ Second-line treatment includes methotrexate, mycophenolate mofetil, systemic retinoids, and azathioprine. Other immunosuppressive agents that are less commonly used include clofazimine, cyclophosphamide, and rituximab.¹

The patient was treated sequentially with trials of oral azathioprine 50 mg bid, then prednisone 10 mg once daily, and then hydroxychloroquine 400 mg daily, without significant change in her condition. Additionally, topical steroids did not improve the patient’s symptoms. She was subsequently started on rituximab 1000 mg intravenously with a second dose repeated 2 weeks later, and another treatment 6 months after that. One year after her visit to the ED, the patient was experiencing marked improvement in her lesions.

‌

Photo courtesy of Christy Nwankwo BA. Text courtesy of Christy Nwankwo, BA, University of Missouri-Kansas City School of Medicine and Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque

A punch-biopsy was performed on the left second toe where the erythema was the most intense. It demonstrated classic findings for pernio: superficial and deep perivascular lymphocytic inflammation and papillary dermal edema on the acral surface.

Pernio, alternatively known as chilblains, is characterized by erythema, violaceous changes, and swelling at acral sites (especially the toes or fingers). There can also be blistering, pain/tenderness, and itch. Pernio results in an abnormal localized inflammatory response to nonfreezing cold and is more common in damp climates. Pernio may also occur in occupational settings where patients handle frozen food. When a patient presents with the classic findings and consistent history, biopsy is not strictly necessary, but can aid in a definitive diagnosis.

The pathogenesis of pernio is not clearly understood. Inflammation secondary to vasospasm and type I interferon immune response to repeated or chronic cold exposure likely play a significant role. Symptoms can arise within 24 hours of exposure and resolve just as quickly. However, persistent and repeated exposure can also trigger ongoing symptoms that last for weeks.

As with most autoinflammatory conditions, pernio has a proclivity to affect younger women. It also affects children and the elderly. Because it is an inflammatory response to nonfreezing cold temperatures, the disease tends to occur during autumn in patients who live in homes without central heating.

A diagnosis of idiopathic pernio necessitates excluding several other similar, cold-induced entities. These include acrocyanosis (due to erythromelalgia, anorexia, medications), Raynaud phenomenon, cryoglobulinemia, cold urticaria, and chilblain lupus (among others). Pernio tends to lack other clinical findings such as true retiform purpura.

Of note, during the COVID-19 pandemic, physicians identified a spike in the incidence of pernio-like acral eruptions. This phenomenon has been coined “COVID toes.” While the direct temporal and causal relationships between COVID-19 and the observed eruption has not been clearly established, any patient who presents with a new onset pernio-like eruption should receive a COVID-19 test to ensure proper precautions are followed.¹

In our patient, the work-up did not show any evidence of other underlying conditions. As her symptoms were minimal, we provided reassurance and counseling on preventive measures such as keeping her hands and feet warm and dry. In cases where treatment is needed, high-potency topical corticosteroids can be utilized judiciously during flares to decrease local inflammation. (There is minimal concern for adverse effects due to the thicker skin on acral surfaces.) Another treatment option is oral nifedipine (20-60 mg/d). One double-blinded trial showed it can improve symptoms in up to 70% of patients.²

‌

Clinical image courtesy of Jiasen Wang, MD; microscopy image courtesy of Shelly Stepenaskie, MD. Text courtesy of Jiasen Wang, MD, Aimee Smidt, MD, Shelly Stepenaskie, MD, Department of Dermatology, and Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

A punch-biopsy was performed on the left second toe where the erythema was the most intense. It demonstrated classic findings for pernio: superficial and deep perivascular lymphocytic inflammation and papillary dermal edema on the acral surface.

Pernio, alternatively known as chilblains, is characterized by erythema, violaceous changes, and swelling at acral sites (especially the toes or fingers). There can also be blistering, pain/tenderness, and itch. Pernio results in an abnormal localized inflammatory response to nonfreezing cold and is more common in damp climates. Pernio may also occur in occupational settings where patients handle frozen food. When a patient presents with the classic findings and consistent history, biopsy is not strictly necessary, but can aid in a definitive diagnosis.

The pathogenesis of pernio is not clearly understood. Inflammation secondary to vasospasm and type I interferon immune response to repeated or chronic cold exposure likely play a significant role. Symptoms can arise within 24 hours of exposure and resolve just as quickly. However, persistent and repeated exposure can also trigger ongoing symptoms that last for weeks.

As with most autoinflammatory conditions, pernio has a proclivity to affect younger women. It also affects children and the elderly. Because it is an inflammatory response to nonfreezing cold temperatures, the disease tends to occur during autumn in patients who live in homes without central heating.

A diagnosis of idiopathic pernio necessitates excluding several other similar, cold-induced entities. These include acrocyanosis (due to erythromelalgia, anorexia, medications), Raynaud phenomenon, cryoglobulinemia, cold urticaria, and chilblain lupus (among others). Pernio tends to lack other clinical findings such as true retiform purpura.

Of note, during the COVID-19 pandemic, physicians identified a spike in the incidence of pernio-like acral eruptions. This phenomenon has been coined “COVID toes.” While the direct temporal and causal relationships between COVID-19 and the observed eruption has not been clearly established, any patient who presents with a new onset pernio-like eruption should receive a COVID-19 test to ensure proper precautions are followed.¹

In our patient, the work-up did not show any evidence of other underlying conditions. As her symptoms were minimal, we provided reassurance and counseling on preventive measures such as keeping her hands and feet warm and dry. In cases where treatment is needed, high-potency topical corticosteroids can be utilized judiciously during flares to decrease local inflammation. (There is minimal concern for adverse effects due to the thicker skin on acral surfaces.) Another treatment option is oral nifedipine (20-60 mg/d). One double-blinded trial showed it can improve symptoms in up to 70% of patients.²

‌

Clinical image courtesy of Jiasen Wang, MD; microscopy image courtesy of Shelly Stepenaskie, MD. Text courtesy of Jiasen Wang, MD, Aimee Smidt, MD, Shelly Stepenaskie, MD, Department of Dermatology, and Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

A punch-biopsy was performed on the left second toe where the erythema was the most intense. It demonstrated classic findings for pernio: superficial and deep perivascular lymphocytic inflammation and papillary dermal edema on the acral surface.

Pernio, alternatively known as chilblains, is characterized by erythema, violaceous changes, and swelling at acral sites (especially the toes or fingers). There can also be blistering, pain/tenderness, and itch. Pernio results in an abnormal localized inflammatory response to nonfreezing cold and is more common in damp climates. Pernio may also occur in occupational settings where patients handle frozen food. When a patient presents with the classic findings and consistent history, biopsy is not strictly necessary, but can aid in a definitive diagnosis.

The pathogenesis of pernio is not clearly understood. Inflammation secondary to vasospasm and type I interferon immune response to repeated or chronic cold exposure likely play a significant role. Symptoms can arise within 24 hours of exposure and resolve just as quickly. However, persistent and repeated exposure can also trigger ongoing symptoms that last for weeks.

As with most autoinflammatory conditions, pernio has a proclivity to affect younger women. It also affects children and the elderly. Because it is an inflammatory response to nonfreezing cold temperatures, the disease tends to occur during autumn in patients who live in homes without central heating.

A diagnosis of idiopathic pernio necessitates excluding several other similar, cold-induced entities. These include acrocyanosis (due to erythromelalgia, anorexia, medications), Raynaud phenomenon, cryoglobulinemia, cold urticaria, and chilblain lupus (among others). Pernio tends to lack other clinical findings such as true retiform purpura.

Of note, during the COVID-19 pandemic, physicians identified a spike in the incidence of pernio-like acral eruptions. This phenomenon has been coined “COVID toes.” While the direct temporal and causal relationships between COVID-19 and the observed eruption has not been clearly established, any patient who presents with a new onset pernio-like eruption should receive a COVID-19 test to ensure proper precautions are followed.¹

In our patient, the work-up did not show any evidence of other underlying conditions. As her symptoms were minimal, we provided reassurance and counseling on preventive measures such as keeping her hands and feet warm and dry. In cases where treatment is needed, high-potency topical corticosteroids can be utilized judiciously during flares to decrease local inflammation. (There is minimal concern for adverse effects due to the thicker skin on acral surfaces.) Another treatment option is oral nifedipine (20-60 mg/d). One double-blinded trial showed it can improve symptoms in up to 70% of patients.²

‌

Clinical image courtesy of Jiasen Wang, MD; microscopy image courtesy of Shelly Stepenaskie, MD. Text courtesy of Jiasen Wang, MD, Aimee Smidt, MD, Shelly Stepenaskie, MD, Department of Dermatology, and Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

THE PRESENTATION

A Early central centrifugal cicatricial alopecia with a small central patch of hair loss in a 45-year-old Black woman.

B Late central centrifugal cicatricial alopecia with a large central patch of hair loss in a 43-year-old Black woman.

Scarring alopecia is a collection of hair loss disorders including chronic cutaneous lupus erythematosus (discoid lupus), lichen planopilaris, dissecting cellulitis, acne keloidalis, and central centrifugal cicatricial alopecia.¹ CCCA (formerly hot comb alopecia or follicular degeneration syndrome) is a progressive, scarring, inflammatory alopecia and represents the most common form of scarring alopecia in women of African descent. It results in permanent destruction of hair follicles.

Epidemiology

CCCA predominantly affects women of African descent but also may affect men. The prevalence of CCCA in those of African descent has varied in the literature. Khumalo² reported a prevalence of 1.2% for women younger than 50 years and 6.7% in women older than 50 years. CCCA has been reported in other ethnic groups, such as those of Asian descent.³

Historically, hair care practices that are more common in those of African descent, such as high-tension hairstyles as well as heat and chemical hair relaxers, were implicated in the development of CCCA. However, the causes of CCCA are most likely multifactorial, including family history, genetic mutations, and hair care practices.^4-7PADI3 mutations likely predispose some women to CCCA. Mutations in PADI3, which encodes peptidyl arginine deiminase 3 (an enzyme that modifies proteins crucial for the formation of hair shafts), were found in some patients with CCCA.⁸ Moreover, other genetic defects also likely play a role.⁷

Key clinical features

Early recognition is key for patients with CCCA.

• CCCA begins in the central scalp (crown area, vertex) and spreads centrifugally.
• Scalp symptoms such as tenderness, pain, a tingling or crawling sensation, and itching may occur.⁹ Some patients may not have any symptoms at all, and hair loss may progress painlessly.
• Central hair breakage—forme fruste CCCA—may be a presenting sign of CCCA.⁹
• Loss of follicular ostia and mottled hypopigmented and hyperpigmented macules are common findings.⁶
• CCCA can be diagnosed clinically and by histopathology.

Worth noting

Patients may experience hair loss and scalp symptoms for years before seeking medical evaluation. In some cultures, hair breakage or itching on the top of the scalp may be viewed as a normal occurrence in life.

It is important to set patient expectations that CCCA is a scarring alopecia, and the initial goal often is to maintain the patient's existing hair. However, hair and areas responding to treatment should still be treated. Without any intervention, the resulting scarring from CCCA may permanently scar follicles on the entire scalp.

Continue to: Due to the inflammatory...

Due to the inflammatory nature of CCCA, potent topical corticosteroids (eg, clobetasol propionate), intralesional corticosteroids (eg, triamcinolone acetonide), and oral antiinflammatory agents (eg, doxycycline) are utilized in the treatment of CCCA. Minoxidil is another treatment option. Adjuvant therapies such as topical metformin also have been tried.10 Importantly, treatment of CCCA may halt further permanent destruction of hair follicles, but scalp symptoms may reappear periodically and require re-treatment with anti-inflammatory agents.

Health care highlight

Thorough scalp examination and awareness of clinical features of CCCA may prompt earlier diagnosis and prevent future severe permanent alopecia. Clinicians should encourage patients with suggestive signs or symptoms of CCCA to seek care from a dermatologist.

THE PRESENTATION

A Early central centrifugal cicatricial alopecia with a small central patch of hair loss in a 45-year-old Black woman.

B Late central centrifugal cicatricial alopecia with a large central patch of hair loss in a 43-year-old Black woman.

Scarring alopecia is a collection of hair loss disorders including chronic cutaneous lupus erythematosus (discoid lupus), lichen planopilaris, dissecting cellulitis, acne keloidalis, and central centrifugal cicatricial alopecia.¹ CCCA (formerly hot comb alopecia or follicular degeneration syndrome) is a progressive, scarring, inflammatory alopecia and represents the most common form of scarring alopecia in women of African descent. It results in permanent destruction of hair follicles.

Epidemiology

CCCA predominantly affects women of African descent but also may affect men. The prevalence of CCCA in those of African descent has varied in the literature. Khumalo² reported a prevalence of 1.2% for women younger than 50 years and 6.7% in women older than 50 years. CCCA has been reported in other ethnic groups, such as those of Asian descent.³

Historically, hair care practices that are more common in those of African descent, such as high-tension hairstyles as well as heat and chemical hair relaxers, were implicated in the development of CCCA. However, the causes of CCCA are most likely multifactorial, including family history, genetic mutations, and hair care practices.^4-7PADI3 mutations likely predispose some women to CCCA. Mutations in PADI3, which encodes peptidyl arginine deiminase 3 (an enzyme that modifies proteins crucial for the formation of hair shafts), were found in some patients with CCCA.⁸ Moreover, other genetic defects also likely play a role.⁷

Key clinical features

Early recognition is key for patients with CCCA.

• CCCA begins in the central scalp (crown area, vertex) and spreads centrifugally.
• Scalp symptoms such as tenderness, pain, a tingling or crawling sensation, and itching may occur.⁹ Some patients may not have any symptoms at all, and hair loss may progress painlessly.
• Central hair breakage—forme fruste CCCA—may be a presenting sign of CCCA.⁹
• Loss of follicular ostia and mottled hypopigmented and hyperpigmented macules are common findings.⁶
• CCCA can be diagnosed clinically and by histopathology.

Worth noting

Patients may experience hair loss and scalp symptoms for years before seeking medical evaluation. In some cultures, hair breakage or itching on the top of the scalp may be viewed as a normal occurrence in life.

It is important to set patient expectations that CCCA is a scarring alopecia, and the initial goal often is to maintain the patient's existing hair. However, hair and areas responding to treatment should still be treated. Without any intervention, the resulting scarring from CCCA may permanently scar follicles on the entire scalp.

Continue to: Due to the inflammatory...

Due to the inflammatory nature of CCCA, potent topical corticosteroids (eg, clobetasol propionate), intralesional corticosteroids (eg, triamcinolone acetonide), and oral antiinflammatory agents (eg, doxycycline) are utilized in the treatment of CCCA. Minoxidil is another treatment option. Adjuvant therapies such as topical metformin also have been tried.10 Importantly, treatment of CCCA may halt further permanent destruction of hair follicles, but scalp symptoms may reappear periodically and require re-treatment with anti-inflammatory agents.

Health care highlight

Thorough scalp examination and awareness of clinical features of CCCA may prompt earlier diagnosis and prevent future severe permanent alopecia. Clinicians should encourage patients with suggestive signs or symptoms of CCCA to seek care from a dermatologist.

THE PRESENTATION

A Early central centrifugal cicatricial alopecia with a small central patch of hair loss in a 45-year-old Black woman.

B Late central centrifugal cicatricial alopecia with a large central patch of hair loss in a 43-year-old Black woman.

Scarring alopecia is a collection of hair loss disorders including chronic cutaneous lupus erythematosus (discoid lupus), lichen planopilaris, dissecting cellulitis, acne keloidalis, and central centrifugal cicatricial alopecia.¹ CCCA (formerly hot comb alopecia or follicular degeneration syndrome) is a progressive, scarring, inflammatory alopecia and represents the most common form of scarring alopecia in women of African descent. It results in permanent destruction of hair follicles.

Epidemiology

CCCA predominantly affects women of African descent but also may affect men. The prevalence of CCCA in those of African descent has varied in the literature. Khumalo² reported a prevalence of 1.2% for women younger than 50 years and 6.7% in women older than 50 years. CCCA has been reported in other ethnic groups, such as those of Asian descent.³

Historically, hair care practices that are more common in those of African descent, such as high-tension hairstyles as well as heat and chemical hair relaxers, were implicated in the development of CCCA. However, the causes of CCCA are most likely multifactorial, including family history, genetic mutations, and hair care practices.^4-7PADI3 mutations likely predispose some women to CCCA. Mutations in PADI3, which encodes peptidyl arginine deiminase 3 (an enzyme that modifies proteins crucial for the formation of hair shafts), were found in some patients with CCCA.⁸ Moreover, other genetic defects also likely play a role.⁷

Key clinical features

Early recognition is key for patients with CCCA.

• CCCA begins in the central scalp (crown area, vertex) and spreads centrifugally.
• Scalp symptoms such as tenderness, pain, a tingling or crawling sensation, and itching may occur.⁹ Some patients may not have any symptoms at all, and hair loss may progress painlessly.
• Central hair breakage—forme fruste CCCA—may be a presenting sign of CCCA.⁹
• Loss of follicular ostia and mottled hypopigmented and hyperpigmented macules are common findings.⁶
• CCCA can be diagnosed clinically and by histopathology.

Worth noting

Patients may experience hair loss and scalp symptoms for years before seeking medical evaluation. In some cultures, hair breakage or itching on the top of the scalp may be viewed as a normal occurrence in life.

It is important to set patient expectations that CCCA is a scarring alopecia, and the initial goal often is to maintain the patient's existing hair. However, hair and areas responding to treatment should still be treated. Without any intervention, the resulting scarring from CCCA may permanently scar follicles on the entire scalp.

Continue to: Due to the inflammatory...

Due to the inflammatory nature of CCCA, potent topical corticosteroids (eg, clobetasol propionate), intralesional corticosteroids (eg, triamcinolone acetonide), and oral antiinflammatory agents (eg, doxycycline) are utilized in the treatment of CCCA. Minoxidil is another treatment option. Adjuvant therapies such as topical metformin also have been tried.10 Importantly, treatment of CCCA may halt further permanent destruction of hair follicles, but scalp symptoms may reappear periodically and require re-treatment with anti-inflammatory agents.

Health care highlight

Thorough scalp examination and awareness of clinical features of CCCA may prompt earlier diagnosis and prevent future severe permanent alopecia. Clinicians should encourage patients with suggestive signs or symptoms of CCCA to seek care from a dermatologist.

THE CASE

A 52-year-old man presented to the emergency department after vomiting a large volume of blood and was admitted to the intensive care unit. His past medical history was remarkable for untreated chronic hepatitis C resulting from injection drug use and cirrhosis without prior history of esophageal varices.

Due to ongoing hematemesis, he was intubated for airway protection and underwent esophagogastroduodenoscopy with banding of large esophageal varices on hospital day (HD) 1. He was extubated on HD 2 after clinical stability was achieved; however, he became encephalopathic over the subsequent days despite treatment with lactulose. On HD 4, the patient required re-intubation for progressive respiratory failure. Chest imaging revealed a large, simple-appearing right pleural effusion and extensive bilateral patchy ground-glass opacities (FIGURE 1).

Thoracentesis was ordered and revealed transudative pleural fluid; this finding, along with negative infectious studies, was consistent with hepatic hydrothorax. In the setting of initial decompensation, empiric treatment with vancomycin and meropenem was started for suspected hospital-acquired pneumonia.

The patient had persistent fevers that had developed during his hospital stay and pulmonary opacities, despite 72 hours of treatment with broad-spectrum antibiotics. Thus, a diagnostic bronchoscopy with bronchoalveolar lavage (BAL) was performed. BAL cell count and differential revealed 363 nucleated cells/µL, with profound eosinophilia (42% eosinophils, 44% macrophages, 14% neutrophils).

Bacterial and fungal cultures and a viral polymerase chain reaction panel were negative. HIV antibody-antigen and RNA testing were also negative. The patient had no evidence or history of underlying malignancy, autoimmune disease, or recent immunosuppressive therapy, including corticosteroids. Due to consistent imaging findings and lack of improvement with appropriate treatment for bacterial pneumonia, further work-up was pursued.

THE DIAGNOSIS

Given the consistent radiographic pattern, the differential diagnosis for this patient included pneumocystis pneumonia (PCP), a potentially life-threatening opportunistic infection. Work-up therefore included direct fluorescent antibody testing, which was positive for Pneumocystis jirovecii, a fungus that can cause PCP.

Of note, the patient’s white blood cell count was elevated on admission (11.44 × 10³/µL) but low for much of his hospital stay (nadir = 1.97 × 10³/µL), with associated lymphopenia (nadir = 0.22 × 10³/µl). No peripheral eosinophilia was noted.

Continue to: DISCUSSION

PCP typically occurs in immunocompromised individuals and may be related to HIV infection, malignancy, or exposure to immunosuppressive therapies.^1,2 While rare cases of PCP have been described in adults without predisposing factors, many of these cases occurred at the beginning of the AIDS epidemic, prior to reliable HIV testing.^3-5

Uncharted territory. We were confident in our diagnosis because immunofluorescence testing has very few false-positives and a high specificity.^6-8 But there were informational gaps. The eosinophilia recorded on BAL is poorly described in HIV-negative patients with PCP but well-described in HIV-positive patients, with the level of eosinophilia associated with disease severity.^9,10 Eosinophils are thought to contribute to pulmonary inflammation, which may explain the severity of our patient’s course.¹⁰

A first of its kind case?

To our knowledge, this is the first report of PCP in a patient with cirrhosis from chronic hepatitis C virus infection and no other predisposing conditions or preceding immunosuppressive therapy. We suspect that his lymphopenia, which was noted during his critical illness, predisposed him to PCP.

Individuals with lymphopenia and low CD4+ T-cell counts have been shown to be at increased risk of pneumocystis pneumonia.

Lymphocytes (in particular CD4+ T cells) have been shown to play an important role, along with alveolar macrophages and neutrophils, in directing the host defense against P jirovecii infection.^1,3,11 Individuals with lymphopenia and low CD4+ T-cell counts have been shown to be at increased risk of PCP; risk increases markedly with CD4+ T cells below 200 cells/µL.^11-13

Typical risk factors for lymphopenia had not been observed in this patient. However, cirrhosis has been associated with low CD4+ T-cell counts and disruption of cell-mediated immunity, even in HIV-­seronegative patients.^14,15 There are several postulated mechanisms for low CD4+ T-cell counts in cirrhosis, including splenic sequestration, impaired T-cell production (due to impaired thymopoiesis), increased T-cell consumption, and apoptosis (due to persistent immune system activation from bacterial translocation and an overall pro-infl­ammatory state).^16,17

Continue to: Predisposing factors guide treatment

Routine treatment for PCP in patients without HIV is a 21-day course of trimethoprim/­sulfamethoxazole (Bactrim). Dosing for patients with normal renal function is 15 to 20 mg/kg orally or intravenously per day. Patients with allergy to trimethoprim/sulfamethoxazole should ideally undergo desensitization, given its effectiveness against PCP.

Due to a sulfonamide allergy, our patient was started on primaquine 30 mg/d, clindamycin 600 mg tid, and prednisone 40 mg bid. (The corticosteroid was added because of the severity of the disease.) Three days after starting treatment—and 10 days into his hospital stay—the patient had significant improvement in his respiratory status and was successfully extubated. He underwent trimethoprim/sulfamethoxazole desensitization and completed a 21-day course of treatment for PCP with complete resolution of respiratory symptoms. Follow-up chest radiograph 2 months later (FIGURE 2) confirmed clearance of opacities.

THE TAKEAWAY

PCP remains a rare disease in patients without the typical immunosuppressive risk factors. However, it should be considered in patients with cirrhosis who develop respiratory failure, especially those with compatible radiographic findings and negative microbiologic evaluation for other, more typical, organisms.

CORRESPONDENCE
Tyler Albert, MD, VA Puget Sound Healthcare System, 1660 South Columbian Way, S-111-Pulm, Seattle, WA 98108; talbert@uw.edu

THE CASE

A 52-year-old man presented to the emergency department after vomiting a large volume of blood and was admitted to the intensive care unit. His past medical history was remarkable for untreated chronic hepatitis C resulting from injection drug use and cirrhosis without prior history of esophageal varices.

Due to ongoing hematemesis, he was intubated for airway protection and underwent esophagogastroduodenoscopy with banding of large esophageal varices on hospital day (HD) 1. He was extubated on HD 2 after clinical stability was achieved; however, he became encephalopathic over the subsequent days despite treatment with lactulose. On HD 4, the patient required re-intubation for progressive respiratory failure. Chest imaging revealed a large, simple-appearing right pleural effusion and extensive bilateral patchy ground-glass opacities (FIGURE 1).

Thoracentesis was ordered and revealed transudative pleural fluid; this finding, along with negative infectious studies, was consistent with hepatic hydrothorax. In the setting of initial decompensation, empiric treatment with vancomycin and meropenem was started for suspected hospital-acquired pneumonia.

The patient had persistent fevers that had developed during his hospital stay and pulmonary opacities, despite 72 hours of treatment with broad-spectrum antibiotics. Thus, a diagnostic bronchoscopy with bronchoalveolar lavage (BAL) was performed. BAL cell count and differential revealed 363 nucleated cells/µL, with profound eosinophilia (42% eosinophils, 44% macrophages, 14% neutrophils).

Bacterial and fungal cultures and a viral polymerase chain reaction panel were negative. HIV antibody-antigen and RNA testing were also negative. The patient had no evidence or history of underlying malignancy, autoimmune disease, or recent immunosuppressive therapy, including corticosteroids. Due to consistent imaging findings and lack of improvement with appropriate treatment for bacterial pneumonia, further work-up was pursued.

THE DIAGNOSIS

Given the consistent radiographic pattern, the differential diagnosis for this patient included pneumocystis pneumonia (PCP), a potentially life-threatening opportunistic infection. Work-up therefore included direct fluorescent antibody testing, which was positive for Pneumocystis jirovecii, a fungus that can cause PCP.

Of note, the patient’s white blood cell count was elevated on admission (11.44 × 10³/µL) but low for much of his hospital stay (nadir = 1.97 × 10³/µL), with associated lymphopenia (nadir = 0.22 × 10³/µl). No peripheral eosinophilia was noted.

Continue to: DISCUSSION

PCP typically occurs in immunocompromised individuals and may be related to HIV infection, malignancy, or exposure to immunosuppressive therapies.^1,2 While rare cases of PCP have been described in adults without predisposing factors, many of these cases occurred at the beginning of the AIDS epidemic, prior to reliable HIV testing.^3-5

Uncharted territory. We were confident in our diagnosis because immunofluorescence testing has very few false-positives and a high specificity.^6-8 But there were informational gaps. The eosinophilia recorded on BAL is poorly described in HIV-negative patients with PCP but well-described in HIV-positive patients, with the level of eosinophilia associated with disease severity.^9,10 Eosinophils are thought to contribute to pulmonary inflammation, which may explain the severity of our patient’s course.¹⁰

A first of its kind case?

To our knowledge, this is the first report of PCP in a patient with cirrhosis from chronic hepatitis C virus infection and no other predisposing conditions or preceding immunosuppressive therapy. We suspect that his lymphopenia, which was noted during his critical illness, predisposed him to PCP.

Individuals with lymphopenia and low CD4+ T-cell counts have been shown to be at increased risk of pneumocystis pneumonia.

Lymphocytes (in particular CD4+ T cells) have been shown to play an important role, along with alveolar macrophages and neutrophils, in directing the host defense against P jirovecii infection.^1,3,11 Individuals with lymphopenia and low CD4+ T-cell counts have been shown to be at increased risk of PCP; risk increases markedly with CD4+ T cells below 200 cells/µL.^11-13

Typical risk factors for lymphopenia had not been observed in this patient. However, cirrhosis has been associated with low CD4+ T-cell counts and disruption of cell-mediated immunity, even in HIV-­seronegative patients.^14,15 There are several postulated mechanisms for low CD4+ T-cell counts in cirrhosis, including splenic sequestration, impaired T-cell production (due to impaired thymopoiesis), increased T-cell consumption, and apoptosis (due to persistent immune system activation from bacterial translocation and an overall pro-infl­ammatory state).^16,17

Continue to: Predisposing factors guide treatment

Routine treatment for PCP in patients without HIV is a 21-day course of trimethoprim/­sulfamethoxazole (Bactrim). Dosing for patients with normal renal function is 15 to 20 mg/kg orally or intravenously per day. Patients with allergy to trimethoprim/sulfamethoxazole should ideally undergo desensitization, given its effectiveness against PCP.

Due to a sulfonamide allergy, our patient was started on primaquine 30 mg/d, clindamycin 600 mg tid, and prednisone 40 mg bid. (The corticosteroid was added because of the severity of the disease.) Three days after starting treatment—and 10 days into his hospital stay—the patient had significant improvement in his respiratory status and was successfully extubated. He underwent trimethoprim/sulfamethoxazole desensitization and completed a 21-day course of treatment for PCP with complete resolution of respiratory symptoms. Follow-up chest radiograph 2 months later (FIGURE 2) confirmed clearance of opacities.

THE TAKEAWAY

PCP remains a rare disease in patients without the typical immunosuppressive risk factors. However, it should be considered in patients with cirrhosis who develop respiratory failure, especially those with compatible radiographic findings and negative microbiologic evaluation for other, more typical, organisms.

CORRESPONDENCE
Tyler Albert, MD, VA Puget Sound Healthcare System, 1660 South Columbian Way, S-111-Pulm, Seattle, WA 98108; talbert@uw.edu

THE CASE

A 52-year-old man presented to the emergency department after vomiting a large volume of blood and was admitted to the intensive care unit. His past medical history was remarkable for untreated chronic hepatitis C resulting from injection drug use and cirrhosis without prior history of esophageal varices.

Due to ongoing hematemesis, he was intubated for airway protection and underwent esophagogastroduodenoscopy with banding of large esophageal varices on hospital day (HD) 1. He was extubated on HD 2 after clinical stability was achieved; however, he became encephalopathic over the subsequent days despite treatment with lactulose. On HD 4, the patient required re-intubation for progressive respiratory failure. Chest imaging revealed a large, simple-appearing right pleural effusion and extensive bilateral patchy ground-glass opacities (FIGURE 1).

Thoracentesis was ordered and revealed transudative pleural fluid; this finding, along with negative infectious studies, was consistent with hepatic hydrothorax. In the setting of initial decompensation, empiric treatment with vancomycin and meropenem was started for suspected hospital-acquired pneumonia.

The patient had persistent fevers that had developed during his hospital stay and pulmonary opacities, despite 72 hours of treatment with broad-spectrum antibiotics. Thus, a diagnostic bronchoscopy with bronchoalveolar lavage (BAL) was performed. BAL cell count and differential revealed 363 nucleated cells/µL, with profound eosinophilia (42% eosinophils, 44% macrophages, 14% neutrophils).

Bacterial and fungal cultures and a viral polymerase chain reaction panel were negative. HIV antibody-antigen and RNA testing were also negative. The patient had no evidence or history of underlying malignancy, autoimmune disease, or recent immunosuppressive therapy, including corticosteroids. Due to consistent imaging findings and lack of improvement with appropriate treatment for bacterial pneumonia, further work-up was pursued.

THE DIAGNOSIS

Given the consistent radiographic pattern, the differential diagnosis for this patient included pneumocystis pneumonia (PCP), a potentially life-threatening opportunistic infection. Work-up therefore included direct fluorescent antibody testing, which was positive for Pneumocystis jirovecii, a fungus that can cause PCP.

Of note, the patient’s white blood cell count was elevated on admission (11.44 × 10³/µL) but low for much of his hospital stay (nadir = 1.97 × 10³/µL), with associated lymphopenia (nadir = 0.22 × 10³/µl). No peripheral eosinophilia was noted.

Continue to: DISCUSSION

PCP typically occurs in immunocompromised individuals and may be related to HIV infection, malignancy, or exposure to immunosuppressive therapies.^1,2 While rare cases of PCP have been described in adults without predisposing factors, many of these cases occurred at the beginning of the AIDS epidemic, prior to reliable HIV testing.^3-5

Uncharted territory. We were confident in our diagnosis because immunofluorescence testing has very few false-positives and a high specificity.^6-8 But there were informational gaps. The eosinophilia recorded on BAL is poorly described in HIV-negative patients with PCP but well-described in HIV-positive patients, with the level of eosinophilia associated with disease severity.^9,10 Eosinophils are thought to contribute to pulmonary inflammation, which may explain the severity of our patient’s course.¹⁰

A first of its kind case?

To our knowledge, this is the first report of PCP in a patient with cirrhosis from chronic hepatitis C virus infection and no other predisposing conditions or preceding immunosuppressive therapy. We suspect that his lymphopenia, which was noted during his critical illness, predisposed him to PCP.

Individuals with lymphopenia and low CD4+ T-cell counts have been shown to be at increased risk of pneumocystis pneumonia.

Lymphocytes (in particular CD4+ T cells) have been shown to play an important role, along with alveolar macrophages and neutrophils, in directing the host defense against P jirovecii infection.^1,3,11 Individuals with lymphopenia and low CD4+ T-cell counts have been shown to be at increased risk of PCP; risk increases markedly with CD4+ T cells below 200 cells/µL.^11-13

Typical risk factors for lymphopenia had not been observed in this patient. However, cirrhosis has been associated with low CD4+ T-cell counts and disruption of cell-mediated immunity, even in HIV-­seronegative patients.^14,15 There are several postulated mechanisms for low CD4+ T-cell counts in cirrhosis, including splenic sequestration, impaired T-cell production (due to impaired thymopoiesis), increased T-cell consumption, and apoptosis (due to persistent immune system activation from bacterial translocation and an overall pro-infl­ammatory state).^16,17

Continue to: Predisposing factors guide treatment

Routine treatment for PCP in patients without HIV is a 21-day course of trimethoprim/­sulfamethoxazole (Bactrim). Dosing for patients with normal renal function is 15 to 20 mg/kg orally or intravenously per day. Patients with allergy to trimethoprim/sulfamethoxazole should ideally undergo desensitization, given its effectiveness against PCP.

Due to a sulfonamide allergy, our patient was started on primaquine 30 mg/d, clindamycin 600 mg tid, and prednisone 40 mg bid. (The corticosteroid was added because of the severity of the disease.) Three days after starting treatment—and 10 days into his hospital stay—the patient had significant improvement in his respiratory status and was successfully extubated. He underwent trimethoprim/sulfamethoxazole desensitization and completed a 21-day course of treatment for PCP with complete resolution of respiratory symptoms. Follow-up chest radiograph 2 months later (FIGURE 2) confirmed clearance of opacities.

THE TAKEAWAY

PCP remains a rare disease in patients without the typical immunosuppressive risk factors. However, it should be considered in patients with cirrhosis who develop respiratory failure, especially those with compatible radiographic findings and negative microbiologic evaluation for other, more typical, organisms.

CORRESPONDENCE
Tyler Albert, MD, VA Puget Sound Healthcare System, 1660 South Columbian Way, S-111-Pulm, Seattle, WA 98108; talbert@uw.edu

THE CASE

James R* is a 30-year-old man who presented for a primary care walk-in visit due to dizziness, 2 days after he visited an emergency department (ED) for the same concern. He reported episodic symptoms lasting seconds to minutes, specifically when lying down. He said he had not fallen or experienced other physical trauma, did not have blurred vision or hearing loss, and was taking no medications. He also reported panic attacks, during which he experienced palpitations, trembling, paresthesia, and fear of dying. He stated that dizziness did not occur exclusively during panic episodes. His medical history was significant for hypertension; however, he reported significant anxiety related to medical visits. All home blood pressure readings he reported were within normal limits. 

Upon examination, the patient had a blood pressure reading of 142/90 mm Hg and no evidence of nystagmus at rest. A neurologic exam was normal and a Dix-Hallpike maneuver reproduced subjective vertigo without nystagmus. Laboratory findings from the patient’s ED visit were negative for troponin and drug use, and blood oxygenation levels were within normal limits. At the time of this current visit, an electrocardiogram was unremarkable, with the exception of some tachycardia.

The presumptive diagnosis was benign paroxysmal positional vertigo (BPPV). An Epley maneuver was performed in the clinic and resulted in minimal symptom improvement. The physician taught Mr. R how to perform the Epley maneuver himself, prescribed a short course of meclizine, and referred him to the integrated mental health care service to address his panic attacks and anxiety.

Over the next few months, Mr. R continued to report significant distress about the dizzy spells, which persisted even after performing the Epley maneuver, and he reported that the meclizine was causing worsening vertigo. He received an ear-nose-and-throat consultation and cognitive behavioral therapy (CBT)–based consultation/interventions. He also reported avoiding multiple activities due to concerns about his dizziness.

● How would you proceed with this patient?

*The patient’s name and other personally identifying information have been changed to protect his identity.

Somatic symptom disorder (SSD) is characterized by one or more physical symptoms associated with “excessive thoughts, feelings, or behaviors that result in distress and/or functional impairment.”¹ Individuals with SSD are preoccupied with symptom-related severity, experience high symptom-related anxiety, or devote significant time and energy to the symptoms or heath concerns. With a diagnosis of SSD, physical symptoms experienced by the patient may or may not be medically explained. The same symptom need not be continuously present as long as the overall symptomatic presentation lasts 6 months or longer.

The specifier “with predominant pain” is used when pain dominates the presentation.¹ Estimated prevalence of SSD in primary care ranges from 5% to 35%.² The true scope of SSD is difficult to assess accurately since research tends to focus on medically unexplained symptoms, rather than excessive symptom-related concerns. Furthermore, terms such as “medically unexplained symptoms” and “functional syndromes” (including fibromyalgia and irritable bowel syndrome) are frequently used when describing SSD.³

One or more factors may contribute to unexplained symptoms: limitations of medical procedures and techniques, partial clinical information, patients’ inability to follow management recommendations, challenges in differential diagnostics, and access-to-care limitations preventing regular care and appropriate diagnostic work up.

What’s important to remember is that it’s the patient’s reaction to physical symptoms, rather than the presence of symptoms per se, that defines SSD.

Considerations in the differential diagnosis

When making a diagnosis of SSD, symptoms cannot:⁴

• be feigned or deliberately produced as in malingering or factitious disorder.
• result from physiologic effects of a substance (eg, intoxication, withdrawal, or adverse medication effects).
• constitute somatic delusions, as occur in psychotic disorders.
• constitute symptoms or deficits affecting voluntary motor or sensory function that are better explained by neurologic, medical, or psychiatric conditions (consider conversion disorder).
• be preoccupations with physical appearance flaws, as in body dysmorphic disorder.
• be accounted for by an anxiety disorder (eg, palpitations associated with panic attacks).

Continue to: Illness anxiety disorder...

Illness anxiety disorder is also characterized by significant health-related concerns; however, physical symptoms are either mild or absent.

Ongoing elevated screening scores for anxiety and depression refractory to interventions may signal somatic symptom disorder.

Possible causes of SSD are varied and complex, including genetic and biological factors, family dynamics, behavioral modeling/learning, personality traits, difficulties with emotional regulation, and awareness.⁵ Patients may present with ongoing requests for symptom explanations, feelings of helplessness, fear of having concerns dismissed, and low motivation for change.³

Aids in supporting a diagnosis of SSD

It’s not appropriate to rely solely on questionnaires to make the diagnosis of SSD. However, brief screening tools are a time-efficient way to capture patients’ experiences and perceptions.⁶ Along with other components of clinical evaluation, brief symptom screens can both support the diagnosis and help in longitudinal symptom assessment.

Patient Health Questionnaire-15 (PHQ-15), developed for self-report screening in primary care, has desirable psychometric properties including appropriate internal reliability; convergent validity with measures of functional status, disability days, and symptom-related burden; and discriminant validity from measures of depressive symptoms.⁷ The PHQ-15 is an open access tool that is available in several languages. The respondent is asked to rate the extent of being bothered by a range of medical symptoms in the proceeding 4 weeks. Total scores range from 0 to 30, with higher scores indicating greater symptom aggravation. Cutoffs of 5, 10, and 15 correspond to mild, moderate, and severe symptom levels.⁸

Somatic Symptom Disorder - B Criteria Scale (SSD-12) aims to capture SSD symptoms in line with Diagnostic and Statistical Manual of Mental Disorders (DSM-5) diagnostic criteria. It assesses cognitive, affective, and behavioral aspects of SSD.⁹ The SSD-12 is copyrighted and its use requires registration and purchase. Cutoffs by age and gender are available. SSD-12 has demonstrated appropriate reliability and validity.⁹

Continue to: Structured Clinical Interview for DSM Disorders

Structured Clinical Interview for DSM Disorders (SCID)^10,11 is perhaps the most rigorous differential diagnostic tool. However, SCID administration requires training and skill; time for administration and cost of the materials may be prohibitive in primary care.

CBT is empirically supported as a treatment approach to medically unexplained symptoms and somatic symptom disorder.

Finally, SSD symptoms are highly associated with depression and anxiety. Ongoing elevated screening scores for anxiety and depression refractory to interventions may indicate the possibility of overlooked SSD. Furthermore, use of SSD screening tools with anxiety and depression screening tools can provide a more comprehensive picture of impairment, as well as symptom progress.

Treatment: Avoid a split approach

Diagnosing and treating SSD can be challenging for physicians who focus on biomedically based approaches in patient care. Additional tests, studies, and prescriptions are likely to fuel (rather than pacify) patients’ concerns, as such steps divert attention from the underlying psychological needs and mechanisms which maintain SSD. Avoid using a split biopsychosocial approach—ie, beginning the inquiry and treatment planning from a biomedical perspective, and then falling back on psychosocial formulation when treatment efforts have been ineffective. Such an approach leads to understandable patient dissatisfaction and can be interpreted by them as the caregiver suggesting that physical symptoms are “all in [their] head.”¹²

These 4 tips can help

1. Use a biopsychosocial formulation when initiating treatment. Be familiar with biopsychosocial factors in SSD and develop a narrative for discussing this formulation with patients. For example: “Mr. R, we are going to use the following [medical tests/studies/medications] to understand the cause of your symptoms and better manage them. We also need to think about the role of stress and distress in your symptoms because these can also be at play with dizziness.” This may be particularly beneficial for a functional disorder, such as chronic pain. Incorporating patient education resources is an important step toward shared understanding (see Hunter Integrated Pain Service for chronic pain educational videos; www.tga.gov.au/chronic-pain-­management-video-resource-brainman¹³).

2. Combine education about pathophysiology with patient-centered interviewing. Significant SSD symptom improvements were noted following a single 30-minute educational session, while motivational interviewing techniques were used to probe patients’ concerns.²

Continue to: Maintain professionalism and good clinical practice

3. Maintain professionalism and good clinical practice. Consider SSD a medical matter and address it accordingly: explore concerns fully, provide evidence-based responses, communicate empathy, and employ objective management strategies.¹⁴

4. Do not overlook the value of the relationship. A recent systematic review concluded that the relationship between the patient and care provider was central to the success of the interventions for symptom reduction.¹⁵

A controversial approach. Pharmacotherapy for SSD is controversial. While several trials of antidepressants and St. John’s wort have been positive and some authors have stated that all classes of antidepressants are effective for SSD, others maintain that questions regarding dosing, treatment duration, and sustainability of improvement have not been sufficiently addressed in research.^16,17

Coordination of care issues

Primary care continues to be the de facto mental health system, and specialty services may be unavailable or declined by patients.¹⁸ CBT delivered in person or online is empirically supported as a treatment approach to medically unexplained symptoms and SSD.^17,19-22

A recent meta-analysis of randomized controlled trials published by Jing and colleagues²³ reported that CBT was effective for SSD symptom reduction, and that treatment gains were maintained 3 to 12 months post treatment. However, concerns about the practical implementation of CBT in primary care were raised because CBT was not shown to be effective in improving social functioning or reducing the number of medical visits. Symptom improvement was maximized with longer durations of treatment (> 10 sessions) and greater session lengths (> 50 minutes). Additionally, Abbass and colleagues²⁴ brought up several methodologic (sampling and analysis) concerns related to Jing et al’s work.

Continue to: Overally, CBT's effect sizes...

Overall, CBT’s effect sizes are small, and patients who are open to biopsychosocial explanations for their symptoms and to receiving psychological services may differ from most patients seen in primary care practices.²¹ Furthermore, mental health providers may hesitate to diagnose SSD because they are concerned about missing a somatic illness.³ Therefore, when coordinating care with mental health providers, it may be beneficial to discuss the treatment approach, assess familiarity with the SSD diagnosis, and closely coordinate and collaborate on the treatment plan.

While physicians cannot be expected to function as psychotherapists, an understanding of CBT and techniques for SSD treatment can be beneficial. Integrated mental health services may hold promise in addressing SSD in primary care. Onsite availability of a behavioral health provider competent in providing evidence-based care can target SSD symptoms and support both patients and physicians.

THE CASE

Mr. R’s treatment course included multiple primary care appointments (scheduled and walk in), ED visits, and specialist visits (ENT/­vestibular rehabilitation). He sought care as symptoms intensified, lasted longer, or occurred in new circumstances. He reported persistent fear of the symptoms and anxiety that serious medical causes had been overlooked. He also described distress associated with vertigo and his anxiety sensitivity (anxiety about being anxious).

While physicians cannot be expected to function as psychotherapists, an understanding of CBT and techniques for somatic symptom disorder treatment can be beneficial.

The behavioral health consultant (BHC; psychologist) and physician talked to the patient about the biopsychosocial antecedents of his condition and the factors that perpetuate the anxiety and stress response. The BHC described the fight/flight/freeze response to the patient and explained its role in the physiologic stress response associated with somatic symptoms and panic. Educational materials (videos and handouts) were also provided to the patient to further illustrate these concepts. The BHC also discussed the role of interoceptive and situational avoidance and active coping (eg, engaging in safe activities); taught the patient relaxation and grounding techniques; and used cognitive disputation aimed at challenging catastrophic symptom interpretations.

The BHC and the patient’s physician established joint treatment goals that included improving functioning, promoting active coping, and decreasing distress associated with symptoms. After the initial medical and BHC visits, both vertigo and anxiety symptoms appeared to abate somewhat, but symptoms have been ongoing and distress and impairment have been variable. The patient’s family physician and BHC continue to work with him to optimize the care plan and treatment goals.

CORRESPONDENCE
Nataliya Pilipenko, PhD, ABPP, Center for Family and Community Medicine, Columbia University Vagelos College of Physicians and Surgeons, 630 West 168th Street, New York, NY 10032; np2615@cumc.columbia.edu

ACKNOWLEDGEMENT
The author thanks Dr. Molly Warren for her collaboration and guidance.

THE CASE

James R* is a 30-year-old man who presented for a primary care walk-in visit due to dizziness, 2 days after he visited an emergency department (ED) for the same concern. He reported episodic symptoms lasting seconds to minutes, specifically when lying down. He said he had not fallen or experienced other physical trauma, did not have blurred vision or hearing loss, and was taking no medications. He also reported panic attacks, during which he experienced palpitations, trembling, paresthesia, and fear of dying. He stated that dizziness did not occur exclusively during panic episodes. His medical history was significant for hypertension; however, he reported significant anxiety related to medical visits. All home blood pressure readings he reported were within normal limits. 

Upon examination, the patient had a blood pressure reading of 142/90 mm Hg and no evidence of nystagmus at rest. A neurologic exam was normal and a Dix-Hallpike maneuver reproduced subjective vertigo without nystagmus. Laboratory findings from the patient’s ED visit were negative for troponin and drug use, and blood oxygenation levels were within normal limits. At the time of this current visit, an electrocardiogram was unremarkable, with the exception of some tachycardia.

The presumptive diagnosis was benign paroxysmal positional vertigo (BPPV). An Epley maneuver was performed in the clinic and resulted in minimal symptom improvement. The physician taught Mr. R how to perform the Epley maneuver himself, prescribed a short course of meclizine, and referred him to the integrated mental health care service to address his panic attacks and anxiety.

Over the next few months, Mr. R continued to report significant distress about the dizzy spells, which persisted even after performing the Epley maneuver, and he reported that the meclizine was causing worsening vertigo. He received an ear-nose-and-throat consultation and cognitive behavioral therapy (CBT)–based consultation/interventions. He also reported avoiding multiple activities due to concerns about his dizziness.

● How would you proceed with this patient?

*The patient’s name and other personally identifying information have been changed to protect his identity.

Somatic symptom disorder (SSD) is characterized by one or more physical symptoms associated with “excessive thoughts, feelings, or behaviors that result in distress and/or functional impairment.”¹ Individuals with SSD are preoccupied with symptom-related severity, experience high symptom-related anxiety, or devote significant time and energy to the symptoms or heath concerns. With a diagnosis of SSD, physical symptoms experienced by the patient may or may not be medically explained. The same symptom need not be continuously present as long as the overall symptomatic presentation lasts 6 months or longer.

The specifier “with predominant pain” is used when pain dominates the presentation.¹ Estimated prevalence of SSD in primary care ranges from 5% to 35%.² The true scope of SSD is difficult to assess accurately since research tends to focus on medically unexplained symptoms, rather than excessive symptom-related concerns. Furthermore, terms such as “medically unexplained symptoms” and “functional syndromes” (including fibromyalgia and irritable bowel syndrome) are frequently used when describing SSD.³

One or more factors may contribute to unexplained symptoms: limitations of medical procedures and techniques, partial clinical information, patients’ inability to follow management recommendations, challenges in differential diagnostics, and access-to-care limitations preventing regular care and appropriate diagnostic work up.

What’s important to remember is that it’s the patient’s reaction to physical symptoms, rather than the presence of symptoms per se, that defines SSD.

Considerations in the differential diagnosis

When making a diagnosis of SSD, symptoms cannot:⁴

• be feigned or deliberately produced as in malingering or factitious disorder.
• result from physiologic effects of a substance (eg, intoxication, withdrawal, or adverse medication effects).
• constitute somatic delusions, as occur in psychotic disorders.
• constitute symptoms or deficits affecting voluntary motor or sensory function that are better explained by neurologic, medical, or psychiatric conditions (consider conversion disorder).
• be preoccupations with physical appearance flaws, as in body dysmorphic disorder.
• be accounted for by an anxiety disorder (eg, palpitations associated with panic attacks).

Continue to: Illness anxiety disorder...

Illness anxiety disorder is also characterized by significant health-related concerns; however, physical symptoms are either mild or absent.

Ongoing elevated screening scores for anxiety and depression refractory to interventions may signal somatic symptom disorder.

Possible causes of SSD are varied and complex, including genetic and biological factors, family dynamics, behavioral modeling/learning, personality traits, difficulties with emotional regulation, and awareness.⁵ Patients may present with ongoing requests for symptom explanations, feelings of helplessness, fear of having concerns dismissed, and low motivation for change.³

Aids in supporting a diagnosis of SSD

It’s not appropriate to rely solely on questionnaires to make the diagnosis of SSD. However, brief screening tools are a time-efficient way to capture patients’ experiences and perceptions.⁶ Along with other components of clinical evaluation, brief symptom screens can both support the diagnosis and help in longitudinal symptom assessment.

Patient Health Questionnaire-15 (PHQ-15), developed for self-report screening in primary care, has desirable psychometric properties including appropriate internal reliability; convergent validity with measures of functional status, disability days, and symptom-related burden; and discriminant validity from measures of depressive symptoms.⁷ The PHQ-15 is an open access tool that is available in several languages. The respondent is asked to rate the extent of being bothered by a range of medical symptoms in the proceeding 4 weeks. Total scores range from 0 to 30, with higher scores indicating greater symptom aggravation. Cutoffs of 5, 10, and 15 correspond to mild, moderate, and severe symptom levels.⁸

Somatic Symptom Disorder - B Criteria Scale (SSD-12) aims to capture SSD symptoms in line with Diagnostic and Statistical Manual of Mental Disorders (DSM-5) diagnostic criteria. It assesses cognitive, affective, and behavioral aspects of SSD.⁹ The SSD-12 is copyrighted and its use requires registration and purchase. Cutoffs by age and gender are available. SSD-12 has demonstrated appropriate reliability and validity.⁹

Continue to: Structured Clinical Interview for DSM Disorders

Structured Clinical Interview for DSM Disorders (SCID)^10,11 is perhaps the most rigorous differential diagnostic tool. However, SCID administration requires training and skill; time for administration and cost of the materials may be prohibitive in primary care.

CBT is empirically supported as a treatment approach to medically unexplained symptoms and somatic symptom disorder.

Finally, SSD symptoms are highly associated with depression and anxiety. Ongoing elevated screening scores for anxiety and depression refractory to interventions may indicate the possibility of overlooked SSD. Furthermore, use of SSD screening tools with anxiety and depression screening tools can provide a more comprehensive picture of impairment, as well as symptom progress.

Treatment: Avoid a split approach

Diagnosing and treating SSD can be challenging for physicians who focus on biomedically based approaches in patient care. Additional tests, studies, and prescriptions are likely to fuel (rather than pacify) patients’ concerns, as such steps divert attention from the underlying psychological needs and mechanisms which maintain SSD. Avoid using a split biopsychosocial approach—ie, beginning the inquiry and treatment planning from a biomedical perspective, and then falling back on psychosocial formulation when treatment efforts have been ineffective. Such an approach leads to understandable patient dissatisfaction and can be interpreted by them as the caregiver suggesting that physical symptoms are “all in [their] head.”¹²

These 4 tips can help

1. Use a biopsychosocial formulation when initiating treatment. Be familiar with biopsychosocial factors in SSD and develop a narrative for discussing this formulation with patients. For example: “Mr. R, we are going to use the following [medical tests/studies/medications] to understand the cause of your symptoms and better manage them. We also need to think about the role of stress and distress in your symptoms because these can also be at play with dizziness.” This may be particularly beneficial for a functional disorder, such as chronic pain. Incorporating patient education resources is an important step toward shared understanding (see Hunter Integrated Pain Service for chronic pain educational videos; www.tga.gov.au/chronic-pain-­management-video-resource-brainman¹³).

2. Combine education about pathophysiology with patient-centered interviewing. Significant SSD symptom improvements were noted following a single 30-minute educational session, while motivational interviewing techniques were used to probe patients’ concerns.²

Continue to: Maintain professionalism and good clinical practice

3. Maintain professionalism and good clinical practice. Consider SSD a medical matter and address it accordingly: explore concerns fully, provide evidence-based responses, communicate empathy, and employ objective management strategies.¹⁴

4. Do not overlook the value of the relationship. A recent systematic review concluded that the relationship between the patient and care provider was central to the success of the interventions for symptom reduction.¹⁵

A controversial approach. Pharmacotherapy for SSD is controversial. While several trials of antidepressants and St. John’s wort have been positive and some authors have stated that all classes of antidepressants are effective for SSD, others maintain that questions regarding dosing, treatment duration, and sustainability of improvement have not been sufficiently addressed in research.^16,17

Coordination of care issues

Primary care continues to be the de facto mental health system, and specialty services may be unavailable or declined by patients.¹⁸ CBT delivered in person or online is empirically supported as a treatment approach to medically unexplained symptoms and SSD.^17,19-22

A recent meta-analysis of randomized controlled trials published by Jing and colleagues²³ reported that CBT was effective for SSD symptom reduction, and that treatment gains were maintained 3 to 12 months post treatment. However, concerns about the practical implementation of CBT in primary care were raised because CBT was not shown to be effective in improving social functioning or reducing the number of medical visits. Symptom improvement was maximized with longer durations of treatment (> 10 sessions) and greater session lengths (> 50 minutes). Additionally, Abbass and colleagues²⁴ brought up several methodologic (sampling and analysis) concerns related to Jing et al’s work.

Continue to: Overally, CBT's effect sizes...

Overall, CBT’s effect sizes are small, and patients who are open to biopsychosocial explanations for their symptoms and to receiving psychological services may differ from most patients seen in primary care practices.²¹ Furthermore, mental health providers may hesitate to diagnose SSD because they are concerned about missing a somatic illness.³ Therefore, when coordinating care with mental health providers, it may be beneficial to discuss the treatment approach, assess familiarity with the SSD diagnosis, and closely coordinate and collaborate on the treatment plan.

While physicians cannot be expected to function as psychotherapists, an understanding of CBT and techniques for SSD treatment can be beneficial. Integrated mental health services may hold promise in addressing SSD in primary care. Onsite availability of a behavioral health provider competent in providing evidence-based care can target SSD symptoms and support both patients and physicians.

THE CASE

Mr. R’s treatment course included multiple primary care appointments (scheduled and walk in), ED visits, and specialist visits (ENT/­vestibular rehabilitation). He sought care as symptoms intensified, lasted longer, or occurred in new circumstances. He reported persistent fear of the symptoms and anxiety that serious medical causes had been overlooked. He also described distress associated with vertigo and his anxiety sensitivity (anxiety about being anxious).

While physicians cannot be expected to function as psychotherapists, an understanding of CBT and techniques for somatic symptom disorder treatment can be beneficial.

The behavioral health consultant (BHC; psychologist) and physician talked to the patient about the biopsychosocial antecedents of his condition and the factors that perpetuate the anxiety and stress response. The BHC described the fight/flight/freeze response to the patient and explained its role in the physiologic stress response associated with somatic symptoms and panic. Educational materials (videos and handouts) were also provided to the patient to further illustrate these concepts. The BHC also discussed the role of interoceptive and situational avoidance and active coping (eg, engaging in safe activities); taught the patient relaxation and grounding techniques; and used cognitive disputation aimed at challenging catastrophic symptom interpretations.

The BHC and the patient’s physician established joint treatment goals that included improving functioning, promoting active coping, and decreasing distress associated with symptoms. After the initial medical and BHC visits, both vertigo and anxiety symptoms appeared to abate somewhat, but symptoms have been ongoing and distress and impairment have been variable. The patient’s family physician and BHC continue to work with him to optimize the care plan and treatment goals.

CORRESPONDENCE
Nataliya Pilipenko, PhD, ABPP, Center for Family and Community Medicine, Columbia University Vagelos College of Physicians and Surgeons, 630 West 168th Street, New York, NY 10032; np2615@cumc.columbia.edu

ACKNOWLEDGEMENT
The author thanks Dr. Molly Warren for her collaboration and guidance.

THE CASE

James R* is a 30-year-old man who presented for a primary care walk-in visit due to dizziness, 2 days after he visited an emergency department (ED) for the same concern. He reported episodic symptoms lasting seconds to minutes, specifically when lying down. He said he had not fallen or experienced other physical trauma, did not have blurred vision or hearing loss, and was taking no medications. He also reported panic attacks, during which he experienced palpitations, trembling, paresthesia, and fear of dying. He stated that dizziness did not occur exclusively during panic episodes. His medical history was significant for hypertension; however, he reported significant anxiety related to medical visits. All home blood pressure readings he reported were within normal limits. 

Upon examination, the patient had a blood pressure reading of 142/90 mm Hg and no evidence of nystagmus at rest. A neurologic exam was normal and a Dix-Hallpike maneuver reproduced subjective vertigo without nystagmus. Laboratory findings from the patient’s ED visit were negative for troponin and drug use, and blood oxygenation levels were within normal limits. At the time of this current visit, an electrocardiogram was unremarkable, with the exception of some tachycardia.

The presumptive diagnosis was benign paroxysmal positional vertigo (BPPV). An Epley maneuver was performed in the clinic and resulted in minimal symptom improvement. The physician taught Mr. R how to perform the Epley maneuver himself, prescribed a short course of meclizine, and referred him to the integrated mental health care service to address his panic attacks and anxiety.

Over the next few months, Mr. R continued to report significant distress about the dizzy spells, which persisted even after performing the Epley maneuver, and he reported that the meclizine was causing worsening vertigo. He received an ear-nose-and-throat consultation and cognitive behavioral therapy (CBT)–based consultation/interventions. He also reported avoiding multiple activities due to concerns about his dizziness.

● How would you proceed with this patient?

*The patient’s name and other personally identifying information have been changed to protect his identity.

Somatic symptom disorder (SSD) is characterized by one or more physical symptoms associated with “excessive thoughts, feelings, or behaviors that result in distress and/or functional impairment.”¹ Individuals with SSD are preoccupied with symptom-related severity, experience high symptom-related anxiety, or devote significant time and energy to the symptoms or heath concerns. With a diagnosis of SSD, physical symptoms experienced by the patient may or may not be medically explained. The same symptom need not be continuously present as long as the overall symptomatic presentation lasts 6 months or longer.

The specifier “with predominant pain” is used when pain dominates the presentation.¹ Estimated prevalence of SSD in primary care ranges from 5% to 35%.² The true scope of SSD is difficult to assess accurately since research tends to focus on medically unexplained symptoms, rather than excessive symptom-related concerns. Furthermore, terms such as “medically unexplained symptoms” and “functional syndromes” (including fibromyalgia and irritable bowel syndrome) are frequently used when describing SSD.³

One or more factors may contribute to unexplained symptoms: limitations of medical procedures and techniques, partial clinical information, patients’ inability to follow management recommendations, challenges in differential diagnostics, and access-to-care limitations preventing regular care and appropriate diagnostic work up.

What’s important to remember is that it’s the patient’s reaction to physical symptoms, rather than the presence of symptoms per se, that defines SSD.

Considerations in the differential diagnosis

When making a diagnosis of SSD, symptoms cannot:⁴

• be feigned or deliberately produced as in malingering or factitious disorder.
• result from physiologic effects of a substance (eg, intoxication, withdrawal, or adverse medication effects).
• constitute somatic delusions, as occur in psychotic disorders.
• constitute symptoms or deficits affecting voluntary motor or sensory function that are better explained by neurologic, medical, or psychiatric conditions (consider conversion disorder).
• be preoccupations with physical appearance flaws, as in body dysmorphic disorder.
• be accounted for by an anxiety disorder (eg, palpitations associated with panic attacks).

Continue to: Illness anxiety disorder...

Illness anxiety disorder is also characterized by significant health-related concerns; however, physical symptoms are either mild or absent.

Ongoing elevated screening scores for anxiety and depression refractory to interventions may signal somatic symptom disorder.

Possible causes of SSD are varied and complex, including genetic and biological factors, family dynamics, behavioral modeling/learning, personality traits, difficulties with emotional regulation, and awareness.⁵ Patients may present with ongoing requests for symptom explanations, feelings of helplessness, fear of having concerns dismissed, and low motivation for change.³

Aids in supporting a diagnosis of SSD

It’s not appropriate to rely solely on questionnaires to make the diagnosis of SSD. However, brief screening tools are a time-efficient way to capture patients’ experiences and perceptions.⁶ Along with other components of clinical evaluation, brief symptom screens can both support the diagnosis and help in longitudinal symptom assessment.

Patient Health Questionnaire-15 (PHQ-15), developed for self-report screening in primary care, has desirable psychometric properties including appropriate internal reliability; convergent validity with measures of functional status, disability days, and symptom-related burden; and discriminant validity from measures of depressive symptoms.⁷ The PHQ-15 is an open access tool that is available in several languages. The respondent is asked to rate the extent of being bothered by a range of medical symptoms in the proceeding 4 weeks. Total scores range from 0 to 30, with higher scores indicating greater symptom aggravation. Cutoffs of 5, 10, and 15 correspond to mild, moderate, and severe symptom levels.⁸

Somatic Symptom Disorder - B Criteria Scale (SSD-12) aims to capture SSD symptoms in line with Diagnostic and Statistical Manual of Mental Disorders (DSM-5) diagnostic criteria. It assesses cognitive, affective, and behavioral aspects of SSD.⁹ The SSD-12 is copyrighted and its use requires registration and purchase. Cutoffs by age and gender are available. SSD-12 has demonstrated appropriate reliability and validity.⁹

Continue to: Structured Clinical Interview for DSM Disorders

Structured Clinical Interview for DSM Disorders (SCID)^10,11 is perhaps the most rigorous differential diagnostic tool. However, SCID administration requires training and skill; time for administration and cost of the materials may be prohibitive in primary care.

CBT is empirically supported as a treatment approach to medically unexplained symptoms and somatic symptom disorder.

Finally, SSD symptoms are highly associated with depression and anxiety. Ongoing elevated screening scores for anxiety and depression refractory to interventions may indicate the possibility of overlooked SSD. Furthermore, use of SSD screening tools with anxiety and depression screening tools can provide a more comprehensive picture of impairment, as well as symptom progress.

Treatment: Avoid a split approach

Diagnosing and treating SSD can be challenging for physicians who focus on biomedically based approaches in patient care. Additional tests, studies, and prescriptions are likely to fuel (rather than pacify) patients’ concerns, as such steps divert attention from the underlying psychological needs and mechanisms which maintain SSD. Avoid using a split biopsychosocial approach—ie, beginning the inquiry and treatment planning from a biomedical perspective, and then falling back on psychosocial formulation when treatment efforts have been ineffective. Such an approach leads to understandable patient dissatisfaction and can be interpreted by them as the caregiver suggesting that physical symptoms are “all in [their] head.”¹²

These 4 tips can help

1. Use a biopsychosocial formulation when initiating treatment. Be familiar with biopsychosocial factors in SSD and develop a narrative for discussing this formulation with patients. For example: “Mr. R, we are going to use the following [medical tests/studies/medications] to understand the cause of your symptoms and better manage them. We also need to think about the role of stress and distress in your symptoms because these can also be at play with dizziness.” This may be particularly beneficial for a functional disorder, such as chronic pain. Incorporating patient education resources is an important step toward shared understanding (see Hunter Integrated Pain Service for chronic pain educational videos; www.tga.gov.au/chronic-pain-­management-video-resource-brainman¹³).

2. Combine education about pathophysiology with patient-centered interviewing. Significant SSD symptom improvements were noted following a single 30-minute educational session, while motivational interviewing techniques were used to probe patients’ concerns.²

Continue to: Maintain professionalism and good clinical practice

3. Maintain professionalism and good clinical practice. Consider SSD a medical matter and address it accordingly: explore concerns fully, provide evidence-based responses, communicate empathy, and employ objective management strategies.¹⁴

4. Do not overlook the value of the relationship. A recent systematic review concluded that the relationship between the patient and care provider was central to the success of the interventions for symptom reduction.¹⁵

A controversial approach. Pharmacotherapy for SSD is controversial. While several trials of antidepressants and St. John’s wort have been positive and some authors have stated that all classes of antidepressants are effective for SSD, others maintain that questions regarding dosing, treatment duration, and sustainability of improvement have not been sufficiently addressed in research.^16,17

Coordination of care issues

Primary care continues to be the de facto mental health system, and specialty services may be unavailable or declined by patients.¹⁸ CBT delivered in person or online is empirically supported as a treatment approach to medically unexplained symptoms and SSD.^17,19-22

A recent meta-analysis of randomized controlled trials published by Jing and colleagues²³ reported that CBT was effective for SSD symptom reduction, and that treatment gains were maintained 3 to 12 months post treatment. However, concerns about the practical implementation of CBT in primary care were raised because CBT was not shown to be effective in improving social functioning or reducing the number of medical visits. Symptom improvement was maximized with longer durations of treatment (> 10 sessions) and greater session lengths (> 50 minutes). Additionally, Abbass and colleagues²⁴ brought up several methodologic (sampling and analysis) concerns related to Jing et al’s work.

Continue to: Overally, CBT's effect sizes...

Overall, CBT’s effect sizes are small, and patients who are open to biopsychosocial explanations for their symptoms and to receiving psychological services may differ from most patients seen in primary care practices.²¹ Furthermore, mental health providers may hesitate to diagnose SSD because they are concerned about missing a somatic illness.³ Therefore, when coordinating care with mental health providers, it may be beneficial to discuss the treatment approach, assess familiarity with the SSD diagnosis, and closely coordinate and collaborate on the treatment plan.

While physicians cannot be expected to function as psychotherapists, an understanding of CBT and techniques for SSD treatment can be beneficial. Integrated mental health services may hold promise in addressing SSD in primary care. Onsite availability of a behavioral health provider competent in providing evidence-based care can target SSD symptoms and support both patients and physicians.

THE CASE

Mr. R’s treatment course included multiple primary care appointments (scheduled and walk in), ED visits, and specialist visits (ENT/­vestibular rehabilitation). He sought care as symptoms intensified, lasted longer, or occurred in new circumstances. He reported persistent fear of the symptoms and anxiety that serious medical causes had been overlooked. He also described distress associated with vertigo and his anxiety sensitivity (anxiety about being anxious).

While physicians cannot be expected to function as psychotherapists, an understanding of CBT and techniques for somatic symptom disorder treatment can be beneficial.

The behavioral health consultant (BHC; psychologist) and physician talked to the patient about the biopsychosocial antecedents of his condition and the factors that perpetuate the anxiety and stress response. The BHC described the fight/flight/freeze response to the patient and explained its role in the physiologic stress response associated with somatic symptoms and panic. Educational materials (videos and handouts) were also provided to the patient to further illustrate these concepts. The BHC also discussed the role of interoceptive and situational avoidance and active coping (eg, engaging in safe activities); taught the patient relaxation and grounding techniques; and used cognitive disputation aimed at challenging catastrophic symptom interpretations.

The BHC and the patient’s physician established joint treatment goals that included improving functioning, promoting active coping, and decreasing distress associated with symptoms. After the initial medical and BHC visits, both vertigo and anxiety symptoms appeared to abate somewhat, but symptoms have been ongoing and distress and impairment have been variable. The patient’s family physician and BHC continue to work with him to optimize the care plan and treatment goals.

CORRESPONDENCE
Nataliya Pilipenko, PhD, ABPP, Center for Family and Community Medicine, Columbia University Vagelos College of Physicians and Surgeons, 630 West 168th Street, New York, NY 10032; np2615@cumc.columbia.edu

ACKNOWLEDGEMENT
The author thanks Dr. Molly Warren for her collaboration and guidance.

ILLUSTRATIVE CASE

A 16-year-old girl with no chronic medical illness presents to your office with her parents after sustaining a head injury at a soccer game over the weekend. She collided with another player while attempting to head the ball. Immediately afterward, she was taken off the field and assessed. She was confused but had a normal level of consciousness and denied vision changes, weakness or tingling in her arms or legs, severe headache, or neck pain. Further testing revealed dizziness and abnormal balance. Her confusion and abnormal balance resolved after 1 day. She has had a mild headache and light sensitivity since the event. She otherwise feels well at rest in the office. She wants to recover quickly but safely and has heard conflicting statements about whether she should completely rest or start back to light activity now.

Sports-related concussions (SRCs) are highly prevalent in the United States, with as many as 3.8 million cases annually. Of those, 1.1 to 1.9 million cases are in children 18 years old or younger.^2,3 SRCs are defined by the Concussion in Sport Group (CISG) 2017 consensus statement as involving the following criteria: (1) direct or indirect trauma anywhere on the body with force transmitted to the head; (2) rapid or delayed symptom presentation, typically with spontaneous resolution; (3) functional rather than structural injury; and (4) occurrence with or without loss of consciousness with stepwise symptom resolution.⁴

SRCs do not have a proven, effective treatment and can have short- or long-term consequences. Initial treatment includes removing athletes from play immediately after an event. The American Academy of Neurology recommends that athletes not return to play until the concussion is resolved, as judged by a health care provider, and the athlete is asymptomatic when off medication.²

The CISG recommends a 6-step approach, with each step taking at least 24 hours.⁴ The final step is a return to normal activity.⁴ This working group recommended extensive study of rehabilitation programs involving subsymptom threshold exercise (ie, exercise performed at a level that does not exacerbate symptoms) before implementation as routine practice. Evidence from a 2015 study suggests that following strict rest for 5 days until complete symptom resolution may prolong recovery compared with rest for only 1 to 2 days.⁵ Additionally, strict rest did not show a difference in neurocognitive or balance outcomes in that study, and the authors noted it may also negatively impact academic, sports, and social function in adolescents.⁵ This study looked at the potential benefit of subsymptom threshold exercise during recovery from SRC.¹

STUDY SUMMARY

Light aerobic exercise may help speed recovery

This multicenter, prospective, parallel, randomized clinical trial compared subsymptom threshold aerobic exercise to ­placebo-like stretching. Patients were included if they were ages 13 to 18 years and presented within 10 days of an SRC, as diagnosed using the CISG criteria. Exclusion criteria included focal neurologic deficits; history of moderate or severe traumatic brain injury; inability to exercise due to orthopedic injury, cervical spine injury, diabetes, or heart disease; increased cardiac risk; or low postconcussion symptom severity. Patients with a diagnosis of and treatment with medication for ­attention-deficit/hyperactivity disorder (ADHD), depression, anxiety, or learning disorder were excluded, as were patients with a history of more than 3 previous concussions.

It’s unclear whether subsymptom threshold exercise is safe and effective in adolescents with a history of multiple concussions, as those with > 3 concussions were excluded from this study.

Patients in the aerobic exercise group were instructed to use a stationary bike or treadmill (or equivalent walking or jogging if they did not have access to this equipment) at a prescribed heart rate. The target heart rate was 80% of the heart rate achieved during initial assessment with the Buffalo Concussion Treadmill Test (BCTT).⁶ Patients in this group were instructed to exercise for 20 minutes or to the point at which their symptoms increased by 2 points (on a 10-point scale) from pre-exercise levels, whichever came first, with rest prescribed at all other times.

For the placebo-like group, a stretching instruction booklet was provided, with the goal of achieving a heart rate that was not significantly elevated. Participants in this group were told to perform the stretches for 20 minutes daily. Of note, researchers ensured the level of physician and research staff attention was similar for each patient, regardless of treatment group, to prevent intervention bias. Additionally, interventions were not initiated prior to 48 hours from the time of injury.

Continue to: The primary outcome...

The primary outcome was number of days to recovery since the date of injury. This was defined as symptom resolution to normal (as evaluated by a physician blinded to the study group) and by the patient’s ability to exercise to exhaustion without symptom exacerbation on the BCTT. Secondary outcomes measured the proportion of patients with delayed recovery (defined as recovery requiring > 30 days) and daily symptom scores.

Of 165 patients meeting the inclusion criteria, 52 patients were excluded prior to randomization (12 patients chose not to participate, 39 were excluded for lack of symptoms, and 1 withdrew due to severe symptoms on the BCTT). A total of 113 were randomized to either group, and 103 patients completed the study (10 patients did not complete the study or had another illness during the intervention). The study analysis included 52 patients in the aerobic exercise group and 51 in the placebo-like stretching group. The study was powered to detect a significant difference in recovery time.

Patients were about equally divided by sex, with a mean age of 15 years. Patients who had no previous concussion made up 50% of the aerobic group and 57% of the stretching group. The average time since injury was similar in the aerobic and stretching groups (4.9 days and 4.8 days, respectively). The aerobic exercise group recovered in a median of 13 days (interquartile range [IQR] = 10-18.5 days) compared with a median of 17 days (IQR = 13-23 days) for the stretching group (P = .009). The incidence of delayed recovery (> 30 days) was higher in the stretching group (n = 7) compared with the aerobic exercise group (n = 2) but was not statistically significant. Daily symptom reporting occurred at a high rate in both groups, with patients stating that they performed their prescribed exercise 89% of the time. No adverse events were reported.

WHAT’S NEW

First high-quality study to support evidence for early light activity

This is the first high-quality study of subsymptom threshold exercise for SRC. Its findings add to the growing body of evidence that early engagement in light aerobic activity that does not provoke symptoms (but not fully returning to sports activity) can aid in recovery from an SRC.

CAVEATS

Narrow study population limits application of findings

It is unclear if subsymptom threshold exercise is safe and effective in adolescents with a history of multiple concussions, as those with more than 3 concussions were excluded from this study. Additionally, patients with comorbidities such as ADHD, depression, anxiety, or learning disorders were not included in this study, which limits the application of these findings. The generalizability of this study is limited in younger children, adults, those with increased cardiovascular risk, and in patients with concussions that are not sports related.

CHALLENGES TO IMPLEMENTATION

More real-world studies needed to confirm benefit

The majority of adolescent athletes in this study completed the subsymptom threshold exercise in a monitored environment with trainers, heart rate monitors, and access to equipment, limiting the study’s generalizability. Additionally, physicians need to be familiar with the BCTT to assign heart rate goals and assess improvement. The study environment may be feasible for some but not others. Studies evaluating real-world settings with athletes self-monitoring for symptom threshold with stepwise evaluations are needed and may be more broadly applicable.

ACKNOWLEDGEMENT

The PURLs Surveillance System was supported in part by Grant Number UL1RR024999 from the National Center for Research Resources, a Clinical Translational Science Award to the University of Chicago. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Center for Research Resources or the National Institutes of Health.

ILLUSTRATIVE CASE

A 16-year-old girl with no chronic medical illness presents to your office with her parents after sustaining a head injury at a soccer game over the weekend. She collided with another player while attempting to head the ball. Immediately afterward, she was taken off the field and assessed. She was confused but had a normal level of consciousness and denied vision changes, weakness or tingling in her arms or legs, severe headache, or neck pain. Further testing revealed dizziness and abnormal balance. Her confusion and abnormal balance resolved after 1 day. She has had a mild headache and light sensitivity since the event. She otherwise feels well at rest in the office. She wants to recover quickly but safely and has heard conflicting statements about whether she should completely rest or start back to light activity now.

Sports-related concussions (SRCs) are highly prevalent in the United States, with as many as 3.8 million cases annually. Of those, 1.1 to 1.9 million cases are in children 18 years old or younger.^2,3 SRCs are defined by the Concussion in Sport Group (CISG) 2017 consensus statement as involving the following criteria: (1) direct or indirect trauma anywhere on the body with force transmitted to the head; (2) rapid or delayed symptom presentation, typically with spontaneous resolution; (3) functional rather than structural injury; and (4) occurrence with or without loss of consciousness with stepwise symptom resolution.⁴

SRCs do not have a proven, effective treatment and can have short- or long-term consequences. Initial treatment includes removing athletes from play immediately after an event. The American Academy of Neurology recommends that athletes not return to play until the concussion is resolved, as judged by a health care provider, and the athlete is asymptomatic when off medication.²

The CISG recommends a 6-step approach, with each step taking at least 24 hours.⁴ The final step is a return to normal activity.⁴ This working group recommended extensive study of rehabilitation programs involving subsymptom threshold exercise (ie, exercise performed at a level that does not exacerbate symptoms) before implementation as routine practice. Evidence from a 2015 study suggests that following strict rest for 5 days until complete symptom resolution may prolong recovery compared with rest for only 1 to 2 days.⁵ Additionally, strict rest did not show a difference in neurocognitive or balance outcomes in that study, and the authors noted it may also negatively impact academic, sports, and social function in adolescents.⁵ This study looked at the potential benefit of subsymptom threshold exercise during recovery from SRC.¹

STUDY SUMMARY

Light aerobic exercise may help speed recovery

This multicenter, prospective, parallel, randomized clinical trial compared subsymptom threshold aerobic exercise to ­placebo-like stretching. Patients were included if they were ages 13 to 18 years and presented within 10 days of an SRC, as diagnosed using the CISG criteria. Exclusion criteria included focal neurologic deficits; history of moderate or severe traumatic brain injury; inability to exercise due to orthopedic injury, cervical spine injury, diabetes, or heart disease; increased cardiac risk; or low postconcussion symptom severity. Patients with a diagnosis of and treatment with medication for ­attention-deficit/hyperactivity disorder (ADHD), depression, anxiety, or learning disorder were excluded, as were patients with a history of more than 3 previous concussions.

It’s unclear whether subsymptom threshold exercise is safe and effective in adolescents with a history of multiple concussions, as those with > 3 concussions were excluded from this study.

Patients in the aerobic exercise group were instructed to use a stationary bike or treadmill (or equivalent walking or jogging if they did not have access to this equipment) at a prescribed heart rate. The target heart rate was 80% of the heart rate achieved during initial assessment with the Buffalo Concussion Treadmill Test (BCTT).⁶ Patients in this group were instructed to exercise for 20 minutes or to the point at which their symptoms increased by 2 points (on a 10-point scale) from pre-exercise levels, whichever came first, with rest prescribed at all other times.

For the placebo-like group, a stretching instruction booklet was provided, with the goal of achieving a heart rate that was not significantly elevated. Participants in this group were told to perform the stretches for 20 minutes daily. Of note, researchers ensured the level of physician and research staff attention was similar for each patient, regardless of treatment group, to prevent intervention bias. Additionally, interventions were not initiated prior to 48 hours from the time of injury.

Continue to: The primary outcome...

The primary outcome was number of days to recovery since the date of injury. This was defined as symptom resolution to normal (as evaluated by a physician blinded to the study group) and by the patient’s ability to exercise to exhaustion without symptom exacerbation on the BCTT. Secondary outcomes measured the proportion of patients with delayed recovery (defined as recovery requiring > 30 days) and daily symptom scores.

Of 165 patients meeting the inclusion criteria, 52 patients were excluded prior to randomization (12 patients chose not to participate, 39 were excluded for lack of symptoms, and 1 withdrew due to severe symptoms on the BCTT). A total of 113 were randomized to either group, and 103 patients completed the study (10 patients did not complete the study or had another illness during the intervention). The study analysis included 52 patients in the aerobic exercise group and 51 in the placebo-like stretching group. The study was powered to detect a significant difference in recovery time.

Patients were about equally divided by sex, with a mean age of 15 years. Patients who had no previous concussion made up 50% of the aerobic group and 57% of the stretching group. The average time since injury was similar in the aerobic and stretching groups (4.9 days and 4.8 days, respectively). The aerobic exercise group recovered in a median of 13 days (interquartile range [IQR] = 10-18.5 days) compared with a median of 17 days (IQR = 13-23 days) for the stretching group (P = .009). The incidence of delayed recovery (> 30 days) was higher in the stretching group (n = 7) compared with the aerobic exercise group (n = 2) but was not statistically significant. Daily symptom reporting occurred at a high rate in both groups, with patients stating that they performed their prescribed exercise 89% of the time. No adverse events were reported.

WHAT’S NEW

First high-quality study to support evidence for early light activity

This is the first high-quality study of subsymptom threshold exercise for SRC. Its findings add to the growing body of evidence that early engagement in light aerobic activity that does not provoke symptoms (but not fully returning to sports activity) can aid in recovery from an SRC.

CAVEATS

Narrow study population limits application of findings

It is unclear if subsymptom threshold exercise is safe and effective in adolescents with a history of multiple concussions, as those with more than 3 concussions were excluded from this study. Additionally, patients with comorbidities such as ADHD, depression, anxiety, or learning disorders were not included in this study, which limits the application of these findings. The generalizability of this study is limited in younger children, adults, those with increased cardiovascular risk, and in patients with concussions that are not sports related.

CHALLENGES TO IMPLEMENTATION

More real-world studies needed to confirm benefit

The majority of adolescent athletes in this study completed the subsymptom threshold exercise in a monitored environment with trainers, heart rate monitors, and access to equipment, limiting the study’s generalizability. Additionally, physicians need to be familiar with the BCTT to assign heart rate goals and assess improvement. The study environment may be feasible for some but not others. Studies evaluating real-world settings with athletes self-monitoring for symptom threshold with stepwise evaluations are needed and may be more broadly applicable.

ACKNOWLEDGEMENT

The PURLs Surveillance System was supported in part by Grant Number UL1RR024999 from the National Center for Research Resources, a Clinical Translational Science Award to the University of Chicago. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Center for Research Resources or the National Institutes of Health.

ILLUSTRATIVE CASE

A 16-year-old girl with no chronic medical illness presents to your office with her parents after sustaining a head injury at a soccer game over the weekend. She collided with another player while attempting to head the ball. Immediately afterward, she was taken off the field and assessed. She was confused but had a normal level of consciousness and denied vision changes, weakness or tingling in her arms or legs, severe headache, or neck pain. Further testing revealed dizziness and abnormal balance. Her confusion and abnormal balance resolved after 1 day. She has had a mild headache and light sensitivity since the event. She otherwise feels well at rest in the office. She wants to recover quickly but safely and has heard conflicting statements about whether she should completely rest or start back to light activity now.

Sports-related concussions (SRCs) are highly prevalent in the United States, with as many as 3.8 million cases annually. Of those, 1.1 to 1.9 million cases are in children 18 years old or younger.^2,3 SRCs are defined by the Concussion in Sport Group (CISG) 2017 consensus statement as involving the following criteria: (1) direct or indirect trauma anywhere on the body with force transmitted to the head; (2) rapid or delayed symptom presentation, typically with spontaneous resolution; (3) functional rather than structural injury; and (4) occurrence with or without loss of consciousness with stepwise symptom resolution.⁴

SRCs do not have a proven, effective treatment and can have short- or long-term consequences. Initial treatment includes removing athletes from play immediately after an event. The American Academy of Neurology recommends that athletes not return to play until the concussion is resolved, as judged by a health care provider, and the athlete is asymptomatic when off medication.²

The CISG recommends a 6-step approach, with each step taking at least 24 hours.⁴ The final step is a return to normal activity.⁴ This working group recommended extensive study of rehabilitation programs involving subsymptom threshold exercise (ie, exercise performed at a level that does not exacerbate symptoms) before implementation as routine practice. Evidence from a 2015 study suggests that following strict rest for 5 days until complete symptom resolution may prolong recovery compared with rest for only 1 to 2 days.⁵ Additionally, strict rest did not show a difference in neurocognitive or balance outcomes in that study, and the authors noted it may also negatively impact academic, sports, and social function in adolescents.⁵ This study looked at the potential benefit of subsymptom threshold exercise during recovery from SRC.¹

STUDY SUMMARY

Light aerobic exercise may help speed recovery

This multicenter, prospective, parallel, randomized clinical trial compared subsymptom threshold aerobic exercise to ­placebo-like stretching. Patients were included if they were ages 13 to 18 years and presented within 10 days of an SRC, as diagnosed using the CISG criteria. Exclusion criteria included focal neurologic deficits; history of moderate or severe traumatic brain injury; inability to exercise due to orthopedic injury, cervical spine injury, diabetes, or heart disease; increased cardiac risk; or low postconcussion symptom severity. Patients with a diagnosis of and treatment with medication for ­attention-deficit/hyperactivity disorder (ADHD), depression, anxiety, or learning disorder were excluded, as were patients with a history of more than 3 previous concussions.

It’s unclear whether subsymptom threshold exercise is safe and effective in adolescents with a history of multiple concussions, as those with > 3 concussions were excluded from this study.

Patients in the aerobic exercise group were instructed to use a stationary bike or treadmill (or equivalent walking or jogging if they did not have access to this equipment) at a prescribed heart rate. The target heart rate was 80% of the heart rate achieved during initial assessment with the Buffalo Concussion Treadmill Test (BCTT).⁶ Patients in this group were instructed to exercise for 20 minutes or to the point at which their symptoms increased by 2 points (on a 10-point scale) from pre-exercise levels, whichever came first, with rest prescribed at all other times.

For the placebo-like group, a stretching instruction booklet was provided, with the goal of achieving a heart rate that was not significantly elevated. Participants in this group were told to perform the stretches for 20 minutes daily. Of note, researchers ensured the level of physician and research staff attention was similar for each patient, regardless of treatment group, to prevent intervention bias. Additionally, interventions were not initiated prior to 48 hours from the time of injury.

Continue to: The primary outcome...

The primary outcome was number of days to recovery since the date of injury. This was defined as symptom resolution to normal (as evaluated by a physician blinded to the study group) and by the patient’s ability to exercise to exhaustion without symptom exacerbation on the BCTT. Secondary outcomes measured the proportion of patients with delayed recovery (defined as recovery requiring > 30 days) and daily symptom scores.

Of 165 patients meeting the inclusion criteria, 52 patients were excluded prior to randomization (12 patients chose not to participate, 39 were excluded for lack of symptoms, and 1 withdrew due to severe symptoms on the BCTT). A total of 113 were randomized to either group, and 103 patients completed the study (10 patients did not complete the study or had another illness during the intervention). The study analysis included 52 patients in the aerobic exercise group and 51 in the placebo-like stretching group. The study was powered to detect a significant difference in recovery time.

Patients were about equally divided by sex, with a mean age of 15 years. Patients who had no previous concussion made up 50% of the aerobic group and 57% of the stretching group. The average time since injury was similar in the aerobic and stretching groups (4.9 days and 4.8 days, respectively). The aerobic exercise group recovered in a median of 13 days (interquartile range [IQR] = 10-18.5 days) compared with a median of 17 days (IQR = 13-23 days) for the stretching group (P = .009). The incidence of delayed recovery (> 30 days) was higher in the stretching group (n = 7) compared with the aerobic exercise group (n = 2) but was not statistically significant. Daily symptom reporting occurred at a high rate in both groups, with patients stating that they performed their prescribed exercise 89% of the time. No adverse events were reported.

WHAT’S NEW

First high-quality study to support evidence for early light activity

This is the first high-quality study of subsymptom threshold exercise for SRC. Its findings add to the growing body of evidence that early engagement in light aerobic activity that does not provoke symptoms (but not fully returning to sports activity) can aid in recovery from an SRC.

CAVEATS

Narrow study population limits application of findings

It is unclear if subsymptom threshold exercise is safe and effective in adolescents with a history of multiple concussions, as those with more than 3 concussions were excluded from this study. Additionally, patients with comorbidities such as ADHD, depression, anxiety, or learning disorders were not included in this study, which limits the application of these findings. The generalizability of this study is limited in younger children, adults, those with increased cardiovascular risk, and in patients with concussions that are not sports related.

CHALLENGES TO IMPLEMENTATION

More real-world studies needed to confirm benefit

The majority of adolescent athletes in this study completed the subsymptom threshold exercise in a monitored environment with trainers, heart rate monitors, and access to equipment, limiting the study’s generalizability. Additionally, physicians need to be familiar with the BCTT to assign heart rate goals and assess improvement. The study environment may be feasible for some but not others. Studies evaluating real-world settings with athletes self-monitoring for symptom threshold with stepwise evaluations are needed and may be more broadly applicable.

ACKNOWLEDGEMENT

The PURLs Surveillance System was supported in part by Grant Number UL1RR024999 from the National Center for Research Resources, a Clinical Translational Science Award to the University of Chicago. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Center for Research Resources or the National Institutes of Health.