Bolley R, Mistry-Burchardi N, Samtleben W
Schwerpunkt Nephrologie der Medizinischen Klinik I, Klinikum der Ludwig-Maximilians-Universität München-Grosshadern.
Dtsch Med Wochenschr. 2000 Dec 15;125(50):1519-25. doi: 10.1055/s-2000-9478.
Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) are primary necrotizing ANCA associated systemic vasculitides with preferential involvement of small and medium-sized vessels. It was the aim of this study to evaluate retrospectively data regarding diagnostic and clinical features collected over a long period on patients with WG and MPA at one medical centre. Additionally, the question was addressed whether both diseases are being more frequently diagnosed since the introduction of serological tests for ANCA.
The files of all patients with WG (n = 48) and MPA (n = 6) seen between 1976 and 1997 by our nephrology team were evaluated with respect to clinical presentation (signs and symptoms, outcome, complications) and relevant laboratory data (e.g. blood count, ESR, renal function, CRP, ANCA).
Five of 48 patients with WG (24 males, 24 females, 22-67 and 18-77 years) had been diagnosed with the disease in the first 10 years before and 43 after ANCA serology became available. All MPA cases (6 males) had been diagnosed after 1992 and showed renal involvement. The pattern of organ involvement in this cohort with WG was the same as that recorded in the literature. Laboratory tests revealed raised ESR in 85%, anemia in 79%, ANCAs in 91% and increased CRP in 73%. A localized form of WG had been present in 15% of patients, the generalized in 85%. Treatment of the generalized form largely followed the Fauci scheme, achieving remission in 97%. After one year of immunosuppressive treatment 93% of patients with WG were still alive, 74% after 5 years.
This is the first report in Central Europe to have analysed data on patients with WG and MPA, followed up for 20 years at one centre. There were no significant differences in the pattern of organ involvement, results of treatment and prognosis from previously published multicenter studies. Our data demonstrate that the diagnosis of WG has been more frequently made since the introduction of immunological tests for ANCA.
韦格纳肉芽肿（WG）和显微镜下多血管炎（MPA）是原发性坏死性抗中性粒细胞胞浆抗体（ANCA）相关性系统性血管炎，主要累及中小血管。本研究旨在回顾性评估某医疗中心长期收集的关于WG和MPA患者的诊断及临床特征的数据。此外，还探讨了自引入ANCA血清学检测以来，这两种疾病的诊断是否更为频繁。
对1976年至1997年间由我们肾内科团队诊治的所有WG患者（n = 48）和MPA患者（n = 6）的病历进行评估，内容包括临床表现（体征和症状、转归、并发症）以及相关实验室数据（如血细胞计数、血沉、肾功能、CRP、ANCA）。
48例WG患者（24例男性，24例女性，年龄分别为22 - 67岁和18 - 77岁）中，5例在ANCA血清学检测可用之前的前10年被诊断出该病，43例在之后被诊断出。所有MPA病例（6例男性）均在1992年之后被诊断出，且均有肾脏受累。该队列中WG患者的器官受累模式与文献记载一致。实验室检查显示，85%的患者血沉升高，79%的患者贫血，91%的患者ANCA阳性，73%的患者CRP升高。15%的患者为局限性WG，85%为全身性。全身性WG的治疗基本遵循福西方案，缓解率达97%。免疫抑制治疗1年后，93%的WG患者仍存活，5年后为74%。
这是中欧地区首份对WG和MPA患者数据进行分析的报告，在一个中心对患者进行了20年的随访。器官受累模式、治疗结果和预后与之前发表的多中心研究相比无显著差异。我们的数据表明自引入ANCA免疫检测以来，WG的诊断更为频繁。
