All issues > Volume 37(8); 1994

Case Report

J Korean Pediatr Soc. 1994;37(8):1175-1181. Published online August 15, 1994.

A Case of Anti-Neutrophil Cytoplasmic Antibodies (ANCA) Positive Wegener's Granulomatosis

Won Tae WT Kim¹, Woo Jeong WJ Kim¹, Joon Sik JS Kim¹, Chin Moon CM Kang¹, Kwan Kyu KK Park²

¹Department of Pediatrics, Keimyung University, School of Medicine, Taegu, Korea
²Department of Pathology, Keimyung University, School of Medicine, Taegu, Korea

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.

Keywords :Wegener's Granulomatosis, Antineutrophil Cytoplasmic Antibodies, Rapidly Progressive Glomerulonephritis