Clinical aspects of pulmonary sarcoidosis

Pulmonary sarcoidosis has a variable natural course from an asymptomatic state to a progressive life-threatening condition. Radiographic abnormalities are frequently an important clue to the diagnosis. The diagnosis usually requires a tissue biopsy that demonstrates noncaseating granulomas that cannot be ascribed to another clinical condition. The lung may be biopsied, but extrapulmonary sites may be biopsied for the diagnosis if such sites are involved with disease. When the lung is biopsied, a transbronchial lung biopsy with a flexible bronchoscope is the procedure of choice, even if the chest radiograph shows thoracic adenopathy alone without obvious parenchymal infiltration. On occasion the diagnosis can be made on clinical grounds without biopsy when the presentation is highly specific for sarcoidosis, such as Lofgren's Syndrome. Treatment for pulmonary sarcoidosis has not been standardized. Since many patients have spontaneous remissions and the benefits of therapy do not affect the long-term outcome, therapy is reserved for patients with severe or progressive pulmonary symptoms and/or pulmonary dysfunction. Corticosteroids are the primary therapy for pulmonary sarcoidosis. Corticosteroid therapy involves six phases: initial high dose, taper to a maintenance dose, a maintenance dose, taper off corticosteroids, monitor off therapy, and treatment of relapse if it occurs.