Sarcoidosis and systemic vasculitis

Background: Systemic vasculitis is an unusual complication of sarcoidosis. Over a 10-year period, the authors have provided care for six patients who had features of both sarcoidosis and vasculitis. Vasculitis could not be at tributed to other causes. Objectives: To report six patients (five children) who had sarcoidosis and systemic vasculitis and compare our experience with previous literature. To better delineate the clinical spectrum of sarcoid vasculitis and its respo nse to therapy. Methods: Retrospective analysis and a Medline literature review of sarcoid and concurrent vasculitis from 1966. Results: Our six patients had systemic illnesses that included fever, perip heral adenopathy, hilar adenopathy, rash, pulmonary parenchymal disease, mu sculoskeletal symptoms, and scleritis or iridocyclitis. Biopsies revealed f eatures compatible with the diagnosis of sarcoidosis or necrotizing sarcoid granulomata in either skin, lymph node, lung, synovium, bone, bone marrow, liver, trachea, or sclera. Arteriography showed features of large vessel v asculitis in three patients, all of whom were African American, whereas pat ients with small vessel vasculitis were white. prior reports of sarcoid and vasculitis included 14 adults, of whom half had predominantly small vessel disease, and half had medium- or large-sized vessel disease. Eight previou sly reported children included seven with primarily large vessel sarcoid va sculitis. Racial background was noted in 15 reported cases and included whi tes (6), African Americans (5), and Asians (4). Among the authors' six pati ents, four improved when treated with prednisone alone. However, relapses o ccurred when the drug was tapered or withdrawn. Conclusions: Sarcoidosis may be complicated by systemic vasculitis that can affect smalt- to targe-caliber vessels. Sarcoid vasculitis can mimic hyper sensitivity vasculitis, polyarteritis nodosa, microscopic polyangiitis, or Takayasu's arteritis. African American and Asian patients are disproportion ately represented among cases with large vessel involvement. Corticosteroid and cytotoxic therapy is palliative for all forms of sarcoid vasculitis. H owever, relapses and morbidity from disease and treatment is common. Copyri ght (C) 2000 by W.B. Saunders Company.