Wegener's肉芽肿病的临床病理学分析

目的:探讨Wegener's肉芽肿病的临床病理学特点,提高对本病的认识.方法:分析16例Wegener's肉芽肿病患者的临床资料,采用常规病理技术(HE切片、Masson三色染色、PAS染色、PASM染色)与免疫组织化学EnVisionTM法及电子显微镜相结合的方式,观察其组织病理、免疫病理及超微结构形态特征.结果:16例患者中男性5例,女性11例.平均年龄39.4(18～76)岁.从初发到确诊的时间为0.8～48.0(平均16.5)mo.耳、鼻和咽喉部是病程初期最容易受累的部位,累及耳、鼻和喉部者11例(69%);在初发或在整个病程中肺脏受累者14例(87%);出现肾脏损害者3例(19%).最常见的病理学表现为坏死性肉芽肿性炎和血管炎,并以鼻腔黏膜活检组织中最普遍.肾脏主要病理学改变为灶性肾小球肾炎.肾脏超微结构改变为上皮细胞轻度增生、足突融合,毛细血管内皮细胞轻度增生,内皮下少量电子致密物沉积.结论:Wegener's肉芽肿病最重要的组织病理学特点为坏死性肉芽肿性炎和血管炎.其临床表现复杂,确诊困难,需与多种疾病鉴别.临床表现和病理学变化的密切结合是明确诊断的前提,特别是上呼吸道、肺脏和肾脏病理检查是

A clinical and pathological analysis of Wegener's granulomatosis

AIM: To analyze the clinical and pathological features of Wegener's 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's granulomatosis were studied retrospectively. Paraffin-embedded 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 electron-dense deposits of in the subendothelium were the ultrastructural characteristics. CONCLUSION: The most important histopathological features of Wegener's granulomatosis are necrotic granuloma and vasculitis. Wegener's 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.