Hammar S P
Diagnostic Specialties Laboratory, Bremerton, WA 98310, USA.
Semin Respir Infect. 1995 Jun;10(2):107-20.
Vasculitides are classified by the size of the vessel involved and by the nature of the inflammatory process. Pulmonary granulomatous vasculitis encompasses several entities that are in general characterized by granulomatous inflammation, extensive necrosis, and a variegated cellular infiltrate. Wegener's granulomatosis is a prototype of granulomatous vasculitis and is a disease of unknown etiology that often involves the upper respiratory tract, the lower respiratory tract, and the kidneys. Some of the entities initially classified as pulmonary granulomatous vasculitis have subsequently been found to represent other entities; specifically, lymphomas (lymphomatoid granulomatosis) and part of the spectrum of bronchopulmonary aspergillosis (bronchocentric granulomatosis). In addition, it is recognized that certain infectious conditions, specifically the necrotizing inflammatory processes caused by fungi and mycobacteria, can show granulomatous vasculitis and can be confused with Wegener's granulomatosis. The mechanism by which pulmonary granulomatous vasculitis occurs is not well understood, although is thought to have an immunologic basis. A great deal of data has been accumulated concerning antineutrophil cytoplasmic autoantibodies and the role that these antibodies might play in the development of these conditions.
血管炎根据受累血管的大小和炎症过程的性质进行分类。肺部肉芽肿性血管炎包括几种疾病，一般以肉芽肿性炎症、广泛坏死和多样的细胞浸润为特征。韦格纳肉芽肿是肉芽肿性血管炎的一个典型例子，是一种病因不明的疾病，常累及上呼吸道、下呼吸道和肾脏。一些最初被归类为肺部肉芽肿性血管炎的疾病后来被发现代表其他疾病；具体来说，包括淋巴瘤（淋巴瘤样肉芽肿病）和支气管肺曲霉菌病谱系的一部分（支气管中心性肉芽肿病）。此外，人们认识到某些感染性疾病，特别是由真菌和分枝杆菌引起的坏死性炎症过程，可表现为肉芽肿性血管炎，并可能与韦格纳肉芽肿相混淆。尽管认为肺部肉芽肿性血管炎的发病机制有免疫基础，但对其发生机制尚不完全清楚。关于抗中性粒细胞胞浆自身抗体以及这些抗体在这些疾病发展中可能起的作用，已经积累了大量数据。
