Sarcoidosis

Sarcoidosis is a multisystem inflammatory disease characterized by the formation of non-caseating granulomas — small clusters of immune cells — in affected organs. The disease most commonly involves the lungs and lymph nodes, but can affect virtually any organ system, including the skin, eyes, liver, spleen, heart, nervous system, muscles, bones, and joints. The exact cause of sarcoidosis remains unknown, but it is thought to result from an exaggerated immune response to an unidentified environmental trigger in genetically susceptible individuals. It occurs worldwide but disproportionately affects individuals of African descent and Northern European populations, with peak onset typically between 25 and 40 years of age. Key symptoms vary depending on the organs involved. Pulmonary sarcoidosis (ICD-10: D86.0) is the most common form, presenting with persistent dry cough, shortness of breath, and chest discomfort. Bilateral hilar lymphadenopathy is a hallmark radiographic finding. Skin manifestations (D86.3) include erythema nodosum, plaques, papules, and lupus pernio. Ocular involvement (D86.8) may cause uveitis, which can lead to vision impairment if untreated. Cardiac sarcoidosis can cause arrhythmias, heart block, or cardiomyopathy, and neurosarcoidosis may present with cranial nerve palsies, particularly facial nerve palsy. Constitutional symptoms such as fatigue, fever, and weight loss are common. Löfgren syndrome — a distinct acute presentation featuring bilateral hilar lymphadenopathy, erythema nodosum, fever, and polyarthralgia — generally carries an excellent prognosis. Many patients with sarcoidosis experience spontaneous remission, particularly those with Löfgren syndrome or isolated stage I pulmonary disease. For patients requiring treatment, oral corticosteroids (such as prednisone) remain the first-line therapy to suppress granulomatous inflammation. Steroid-sparing immunosuppressive agents, including methotrexate, azathioprine, mycophenolate mofetil, and leflunomide, are used for chronic or refractory disease. Anti-TNF-alpha biologic agents, particularly infliximab, may be considered for severe or treatment-resistant cases. Hydroxychloroquine is sometimes used for skin and joint involvement. Chronic progressive sarcoidosis can lead to pulmonary fibrosis, and in end-stage cases, organ transplantation may be necessary. Regular monitoring of organ function, including pulmonary function tests, cardiac imaging, and ophthalmologic examinations, is essential for optimal disease management.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Sarcoidosis