Sarcoidosis Diagnosis and Treatment

Introduction to Sarcoidosis

• The European Respiratory Society recommends that angiotensin-converting enzyme (ACE) levels should not be used alone for diagnosing sarcoidosis, as they have limited sensitivity (approximately 60%) and can be elevated in other granulomatous conditions 1, 2.
• Elevated ACE levels correlate with inflammatory activity and granulomatous burden, making them useful for monitoring treatment response in patients with initially elevated levels 1, 2, 3.

Cutaneous Sarcoidosis

• Cutaneous sarcoidosis occurs in up to 30% of patients and is often the initial manifestation of the disease 1, 2, 3, 4, 5, 6.
• Specific lesions (with non-caseating granulomas on biopsy) include papules, plaques, subcutaneous nodules, lupus pernio, and cicatricial sarcoidosis 1, 2.

Treatment of Cutaneous Sarcoidosis

• For localized lesions, high-potency topical corticosteroids (clobetasol or halobetasol propionate) should be considered as first-line treatment 7.
• Intralesional injections of triamcinolone acetonide may be more effective than topical preparations for localized lesions 7.
• For patients with cutaneous sarcoidosis and active lesions that cannot be controlled with local treatment, oral corticosteroids should be considered to reduce cutaneous lesions 1, 2, 3, 4, 5, 6.

Systemic Treatment

• Prednisone is the first-line treatment for symptomatic or cosmetically significant cutaneous sarcoidosis 1, 2, 3.
• The response to systemic corticosteroids occurs in up to two-thirds of patients, although effects are often limited to the duration of treatment 1, 2, 3.
• Recurrences are common during corticosteroid tapering, frequently requiring additional immunosuppressive therapy 1, 2, 8.

Alternative Therapies

• Methotrexate is conditionally recommended for symptomatic patients with a high risk of mortality, based on randomized clinical trials 3, 4, 8.
• Infliximab is conditionally recommended for chronic symptomatic pulmonary sarcoidosis that does not respond to other immunosuppressants, including corticosteroids, based on two clinical trials 3, 4, 8.
• Adalimumab has shown efficacy in prospective open studies and small retrospective series 6, 8.

Sarcoidosis Skin Manifestations

Clinical Presentation

• Lupus pernio, a type of specific skin lesion, is characterized by violaceous, indurated plaques typically affecting the nose, cheeks, and ears, according to the Rhinology guideline society 9

Cutaneous Sarcoidosis Treatment

Introduction to Treatment Guidelines

• The European Respiratory Society recommends that patients with cutaneous sarcoidosis and significant skin lesions who do not respond to local treatment should be considered for oral glucocorticoids 10, 11
• For patients with cutaneous sarcoidosis, the European Respiratory Society suggests that treatment should be monitored every 1-2 years, with modifications as needed to prevent toxicity 10, 11

Systemic Treatment Algorithm

• The European Respiratory Society recommends the addition of metotreksat for symptomatic patients who have been treated with glucocorticoids and have ongoing disease, or who have unacceptable side effects from glucocorticoids 10, 11
• The European Respiratory Society suggests that infliximab can be added for patients with cutaneous sarcoidosis who have been treated with glucocorticoids and/or other immunosuppressive agents and have ongoing significant skin disease 10, 11

Evidence‑Based Management of Cutaneous Sarcoidosis

Topical and Intralesional Corticosteroid Therapy

Systemic Corticosteroid Use

Biologic and Immunosuppressive Agents

Negative Findings for Other Agents

Considerations Regarding Long‑Term Corticosteroid Therapy

All facts above are derived from the European Respiratory Journal (2021) and reflect the current evidence base for the treatment of cutaneous sarcoidosis.