Moosig Frank, Lamprecht Peter, Gross Wolfgang L
Department of Rheumatology, Vasculitis Center UKSH & Rheumaklinik Bad Bramstedt, University of Lübeck, Lübeck, Germany.
Clin Rev Allergy Immunol. 2008 Oct;35(1-2):19-21. doi: 10.1007/s12016-007-8067-5.
In the last few years, substantial progress has been achieved in elucidating the pathogenesis of Wegener's granulomatosis. Several genetic risk factors have been described. The structure of the granuloma and its possible function as ectopic lymphoid tissue have been defined. Furthermore, the consecutive immunopathological reactions leading to induction of PR3-antineutrophil cytoplasmic antibodies (ANCA) and the role of ANCA itself is getting clearer. However, the initial events leading to granuloma formation are still widely unknown. Concerning therapy, the significance of the so-called biological agents (TNF-alpha-blockers, Rituximab) still has to be defined.
在过去几年中，韦格纳肉芽肿病的发病机制研究取得了重大进展。已描述了几种遗传危险因素。肉芽肿的结构及其作为异位淋巴组织的可能功能已得到明确。此外，导致抗蛋白酶3-抗中性粒细胞胞浆抗体（ANCA）诱导的连续免疫病理反应以及ANCA本身的作用也越来越清晰。然而，导致肉芽肿形成的初始事件仍然广泛未知。关于治疗，所谓生物制剂（肿瘤坏死因子-α阻滞剂、利妥昔单抗）的意义仍有待确定。
