A clinical and pathological analysis of Wegener¬ðs granulomatosis

    GUO Shuangª²Ping, ZHANG Chuanª²Shan, LIU Yanª²Fang, YANG Li, WANG Linª²Ping, GUO Lin£¬LI Qingª²Long

    1Department of Pathology, Xijing Hospital, Fourth Military Medical University, Xi¬ðan 710033, China, 2Department of Pathology, Ningxian County People¬ðs Hospital, Ningxian 730034, China, 3Department of Pathology, First Hospital of Xianyang, Xianyang 710020, China

    ¡¾Abstract¡¿ AIM: To analyze the clinical and pathological features of Wegener¬ðs granulomatosis so as to improve the management of this disease. METHODS: The clinical and pathological features of 16 (5 males and 11 females) cases of Wegener¬ðs granulomatosis were studied retrospectively. Paraffinª²embedded sections histochemical staining, immunohistochemical staining (EnVisionTM method) and electron microscope were employed in the study. RESULTS: The average age at the onset of the disease was 39.4 (18-76) years and the average time from the onset of clinical symptoms to the diagnosis was 16.5 months. Seven cases were diagnosed within 12 months and 9 cases beyond 12 months. Ear, nose and throat involvements were the most common symptoms (69%) at the beginning of the lesion. The percentages of the lung and renal involvement were 87% and 19% respectively. The most common histopathologic features were mucosal granuloma accompanying vasculitis. The renal lesions were focal segment glomerulonephritis, showing focal proliferation of mesangial cells and endothelium cells, and increased mesangial matrix in some glomeruli. The slight proliferation of epithelium cells and endothelium cells, coalesce of foot process, and a very small amount of electronª²dense deposits of in the subendothelium were the ultrastructural characteristics. CONCLUSION: The most important histopathological features of Wegener¬ðs granulomatosis are necrotic granuloma and vasculitis. Wegener¬ðs granulomatosis is a kind of disease with complicated clinical features and it is necessary to be differentiated from a variety of disease with granulomas. The clinicapathological features are essential for the correct diagnosis, of which the pathological examinations of the upper respiratory tract, the lungs and kidneys are most important. ......