Wegener's Granulomatosis

Abstract

Wegener's granulomatosis is a rare disease of unknown cause that is characterized by necrotizing granulomas of the upper and lower parts of the respiratory tract, necrotizing angiitis, and focal necrotizing glomerulitis. Lesions also are found commonly in the spleen and, indeed, in almost any part of the body, including the heart. Although the condition was first described by Klinger,¹ in 1931, it was not until 1936, when Wegener² reported 3 cases, that the syndrome was recognized as a distinct clinical entity thought to be a variant of periarteritis nodosa. However, in contrast to the latter condition, the presence of hypertension, allergic manifestations, and eosinophilia is rare. After a review of the literature in 1958, Walton³ accepted 56 documented cases of Wegener's granulomatosis, which included his own. Blatt and associates⁴ reviewed the literature since 1897 and found reports of 112 cases of midline granuloma of the upper