Sarcoidosis.

摘要

Sarcoidosis is a systemic granulomatous disease of unknown cause affecting young and middle-aged adults. Patients commonly present with bilateral hilar lymphadenopathy, pulmonary infiltrates, and ocular and skin lesions. The heart, liver,spleen, salivary glands, muscles,bones, kidneys, and central nervous system also may be involved. Diagnosis is based on clinicoradiologic findings plus histologic evidence of noncaseating epithelioid granulomas, and exclusion of other granulomatous diseases. Prognosis correlates with mode of onset, host characteristics, initial clinical course, and extent of disease. The optimal management of sarcoidosis has not been well defined. Although corticosteroids remain the mainstay of treatment, there is little evidence for the optimal initiation, dosage, or duration of therapy. Topical steroids maybe considered for treatment of anterior uveitis and skin lesions. Systemic steroids are indicated for treatment of cardiac, nervous system, severe ocular, and symptomatic or progressive pulmonary involvement. There is little evidence for the efficacy of inhaled steroids. Cytotoxic agents and immunomodulators usually are reserved for treatment of complex or refractory disease. Of these agents, methotrexate is used more frequently because of its safety profile and possible steroid-sparing effects. Antimalarial agents are used frequently for skin lesions, and they have limited success in the treatment of pulmonary disease. Lung and cardiac transplantation is reserved for end-stage disease. Monitoring for symptoms of drug toxicity is essential, and prevention of osteoporosis must be addressed in patients taking long-term oral corticosteroids. It is not known if current therapy alters disease progression.