항호중구 항체(ANCA) 양성의 소아 Wegener 육아종증 1례

Abstract

Wegener's granulomatosis is a disease of unknown etiology that is characterized by the clinicopathologic complex of necrotizing granulomatous vasculitis of the upper and lower respiratory tract, glomerulonephritis, and variable degrees of small vessel vasculitis. Recently Antineutrophil Cytoplasmic Antibody (ANCA) has been reported to be a highly specific test for the diagnosis of Wegener's granulomatosis. We have experienced a patient of Wegener's granulomatosis in a 11 year old girl who was admitted with complaints of arthralgia, hematuria, convulsion and associated with otitis media and sinusitis. Serologic test of C-ANCA was poitive and histologic findings of the kidney showed crescentic glomerulonephritis with sclerosis and surrounding infiltration of multinucleated giant cells. Patient was treated with pulse methylprednisolone without improvement. The clinical course progressed rapidly and expired due to the renal failure, gastrointestinal bleeding and status epilepticus. A brief review of literatures was made.