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1931年Klinger曾首先报告一例有鼻窦破坏,肾炎及播散性脉管炎的病例。1936年Wegeher明确指出这是一种独立的有临床和病理特点的疾病,他描述包括三种主要病变:(1)呼吸道坏死性肉芽肿性病变,(2)全身性病灶性坏死性脉管炎,(3)坏死性肾小球肾炎。上呼吸道最易受累,鼻及鼻腔是好发部位,Wegener最初曾命名“鼻源性肉芽肿”就是根据这个特征。其它部位如鼻窦、眼眶、喉、口腔、皮肤及耳等均可被侵犯。Fahey及Godman于1954年详细地描述了7例病人,并搜集22例过去所报告的病例,从而建立了Wegener氏肉芽肿病(简称WG)的确切诊断标准,氏等指出,典型的全身性WG特点
In 1931, Klinger first reported a case of sinus destruction, nephritis and disseminated vasculitis. In 1936 Wegeher clearly pointed out that this is an independent clinical and pathological features of the disease, he described including three major diseases: (1) respiratory necrotizing granulomatous lesions, (2) systemic focal necrotizing vasculitis , (3) Necrotic glomerulonephritis. Upper respiratory tract most vulnerable to involvement, nasal and nasal cavity is a good site, Wegener was originally named “nasal granuloma” is based on this feature. Other parts such as the sinuses, eyes, throat, mouth, skin and ears can be violated. Fahey and Godman described in detail seven patients in 1954 and collected 22 previously reported cases, establishing the exact diagnostic criteria for Wegener’s granulomatosis (WG), who pointed out that the typical systemic WG Features