本病是Klinger(1931)首次报告的,Wege-ner于1936年报告3例,后来于1939年才认为它是一个独立的临床、病理学综合征。Godman等详细描写了7例病理学资料,并提出以下诊断标准,①上、下呼吸道有坏死性肉芽肿,②可侵犯很多器官以肺为最常见,主要为动、静脉泛发性坏死性脉管炎。③肾球炎并可发展成肉芽肿性肾小球肾炎。Carrington等曾描写一类限于以肺损害为主而无肾球炎的病例,预后略好。Walton回顾了他自己的10例加上选自文献的46例,推断约有83％的患者死于肾衰,其余的大多数在5个月之内死于呼吸衰竭,虽然少数可存活4年。作者提到过去很多努力改善预后的治疗都是令人失望的,但有一些以免疫抑制剂与皮质类固醇等 The disease was first reported by Klinger (1931), and Wegener reported three cases in 1936 and later did not consider it an independent clinical, pathological syndrome in 1939. Godman and other detailed description of seven cases of pathological data, and put forward the following diagnostic criteria, ① upper and lower respiratory tract necrotizing granuloma, ② can invade many organs to the lungs are the most common, mainly for the dynamic venous pannicular necrosis Guan Yan. ③ nephritis and can develop into granulomatous glomerulonephritis. Carrington et al have described a class of cases limited to lung damage without nephritis, with a slightly better prognosis. Walton reviewed 10 of his own cases plus 46 selected from the literature, concluding that about 83% of patients died of renal failure and most of them died of respiratory failure within 5 months, although few survived for 4 years . The authors note that many past efforts to improve the prognosis have been disappointing, but there are some that use immunosuppressive agents such as corticosteroids