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目的:探讨Wegeners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Wegeners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:Wegeners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关键.
Objective: To investigate the clinicopathological features of Wegeners granulomatosis and to improve the understanding of this disease.Methods: The clinical data of 16 patients with Wegeners granulomatosis were analyzed and analyzed by routine pathology (HE section, Masson trichrome staining, PAS staining, PASM staining) and immunohistochemistry EnVisionTM method combined with electron microscopy to observe the histopathological, immunopathological and ultrastructural morphological characteristics.Results: 16 patients, 5 males and 11 females.The average age of 39.4 (18 ~ 76) years old.The time from the initial diagnosis to the diagnosis was 0.8 to 48.0 (average 16.5) mo.The ear, nose and throat were the most vulnerable parts of the initial course of the disease, involving the ear, nose and throat in 11 cases (69% ); 14 cases (87%) of the lungs were affected in the first episode or in the entire course of the disease; 3 cases (19%) of the patients had kidney damage.The most common pathological findings were necrotizing granulomatous inflammation and vasculitis, Nasal mucosa biopsy is the most common renal major pathological changes to focal glomerulonephritis.The renal ultrastructure changes to mild epithelial epithelial cells, foot process fusion, capillary endothelial cell mild hyperplasia, a small amount of electron density under the endothelium Material deposition. Conclusion: Wegeners granuloma The most important histopathological features are necrotizing granulomatous inflammation and vasculitis, the clinical manifestations of which are complex and difficult to diagnose and need to be distinguished from various diseases.The close combination of clinical manifestations and pathological changes is a prerequisite for a definitive diagnosis, in particular Upper respiratory tract, lung and kidney pathology is the key.