Anderson G, Coles E T, Crane M, Douglas A C, Gibbs A R, Geddes D M, Peel E T, Wood J B
Newport Chest Clinic, St. Woolos Hospital, Gwent.
Q J Med. 1992 Jun;83(302):427-38.
In order to describe the British experience of Wegener's granulomatosis Hospital Activity Analysis was used to collect cases diagnosed in England, Wales and Scotland between 1975 and 1985. Where possible clinical details, histological material and chest radiographs were obtained. Two hundred and sixty five patients were considered to have Wegener's granulomatosis. In 109 a single pathologist confirmed the diagnosis by finding both granulomas and vasculitis in biopsy material. The diagnosis was made on clinical grounds or clinical grounds together with histological diagnosis in the local hospital in 156 patients. Wegener's granulomatosis was confined to the lung or upper respiratory tract in 22 per cent of patients and renal disease occurred in 58 per cent. Laboratory tests showed a pattern of mild anaemia, polymorph leucocytosis, eosinophilia and an elevated ESR and hypergammaglobulinaemia, with no specific pattern of changes. Histological confirmation was most frequently obtained by examination of nasal biopsy specimens, but multiple biopsies were often required. Renal biopsies showed focal proliferative glomerulonephritis but granulomatous glomerulonephritis was uncommon. Of available chest radiographs 61 per cent were abnormal, large opacities being most common. Small irregular opacities were found less often and other abnormalities were uncommon. Treatment varied widely and 10 per cent of patients received no drug therapy. This large series illustrates that even without specific treatment, patients with Wegener's granulomatosis can survive for several years and with modern treatment survival for more than a decade is possible. Conclusions about the effectiveness of the various therapies cannot be drawn from this retrospective study. Renal failure and disseminated vasculitis were the commonest causes of death; death was considered to result from complications of treatment with cytotoxic drugs or prednisolone in 6 per cent of patients.
为了描述英国韦格纳肉芽肿病的情况，采用医院活动分析方法收集了1975年至1985年间在英格兰、威尔士和苏格兰确诊的病例。尽可能获取了临床细节、组织学材料和胸部X光片。265名患者被认为患有韦格纳肉芽肿病。其中109例经单一病理学家通过在活检材料中发现肉芽肿和血管炎确诊。156例患者在当地医院根据临床依据或临床依据结合组织学诊断确诊。22%的患者韦格纳肉芽肿病局限于肺部或上呼吸道，58%的患者出现肾脏疾病。实验室检查显示有轻度贫血、多形核白细胞增多、嗜酸性粒细胞增多、血沉升高和高球蛋白血症的表现，但无特定变化模式。组织学确诊最常通过鼻活检标本检查获得，但通常需要多次活检。肾活检显示局灶性增生性肾小球肾炎，但肉芽肿性肾小球肾炎不常见。在现有的胸部X光片中，61%异常，大的阴影最为常见。小的不规则阴影较少见，其他异常不常见。治疗方法差异很大，10%的患者未接受药物治疗。这个大样本系列表明，即使没有特异性治疗，韦格纳肉芽肿病患者也可存活数年，采用现代治疗方法有可能存活超过十年。从这项回顾性研究中无法得出关于各种疗法有效性的结论。肾衰竭和播散性血管炎是最常见的死亡原因；6%的患者死亡被认为是细胞毒性药物或泼尼松龙治疗并发症所致。
