ACR Codes: 6.22
Sarcoidosis is a systemic disease of unknown etiology characterized by noncaseating granulomas found in the lung and other organs. It is slightly more prevalent in women commonly occurring in patients between the ages of 20 and 40 years. This disease is 10 times more prevalent in black Americans. Organs commonly involved include the lungs, skin, lymph nodes, liver, spleen, eyes, joints, central nervous system, and muscles. In the United States the majority of patients present with pulmonary disease, however, a significant number of cases are discovered incidentally on chest x-ray in completely asymptomatic patients. There are two distinct types of sarcoid syndromes with acute presentations: (1) Lofgren syndrome, which presents with erythema nodosum, arthritis, and hilar adenopathy; and (2) Heerfordt-Waldenstrom syndrome, which presents with fever, parotid enlargement, uveitis, and facial palsy. Chest X-ray findings can show the characteristic bilateral hilar adenopathy, reticulonodular parenchyma, honeycombing, and fibrosis. Abnormal laboratory findings include hypercalcemia (caused by an increased production of vitamin D by macrophages), hypercalciuria which can lead to the formation of renal calculi, anemia, and elevated angiotensin-converting enzyme levels. Pulmonary function tests usually suggest a restrictive defect with decreased diffusion capacity and are quite useful for assessing response to treatment. Although there is no evidence of any therapy that alters the course of the disease, corticosteroids can be utilized to treat the acute granulomatous inflammation, hypercalcemia, and extrapulmonary manifestations of the disease involving the eye (iridocyclitis and uveitis), the CNS, bone, and heart.
Reference(s): [1] Andreoli, T.E., Bennett, J.C., Carpenter, C.C. and Plum, F., Cecil Essentials of Medicine, 4th edn., W. B. Saunders Company, Philadelphia, 1997, 143-144 pp.
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Disease Topic 4420 
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