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Sarcoidosis is a multisystem disease that affects the lungs in over 90% of individuals. With an incidence of ~10-64/100, 000 people in the United States and Europe, pulmonary sarcoidosis is one of the most common causes of interstitial lung disease (1). Systemic sarcoidosis is characterized by tremendous heterogeneity in disease manifestations, with variable extra-pulmonary involvement and a clinical course and healthcare burden that vary widely. The diagnosis can be challenging and with uncommon exceptions, requires a confirmatory biopsy and exclusion of competing diagnoses. Pulmonary sarcoidosis may manifest with intra-thoracic lymphadenopathy, pulmonary nodules, infiltrates and/or masses that vary in distribution. Pulmonary hypertension and advanced fibrocystic disease can result in respiratory insufficiency and death. Systemic manifestations such as cardiac, neurologic, abdominal, skin or ocular involvement may dominate the clinical picture and drive treatment approaches (discussed in Chapter 16). We review the scientific basis of sarcoidosis and consensus approaches to manage pulmonary sarcoidosis.

Original publication





Book title

Clinical Handbook of Interstitial Lung Disease

Publication Date



257 - 270