Morović-Vergles Jadranka, Culo Melanie-Ivana, Sutić Anamarija
Lijec Vjesn. 2014 Jul-Aug;136(7-8):228-31.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare diseases, with the average of 30 new cases per million inhabitants per year. Their main characteristic is systemic involvement with necrosis of the vessel walls (histological changes showing necrosis of the media and inflammation of adventitia and intima). In some forms granulomas may be found surrounding the vessels. ANCA-associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, previously called Churg-Straus). Honorific eponyms are now changing to a disease-descriptive or etiology-based nomenclature. ANCA-associated vasculitides are a distinctive group of vasculitides because they dominantly involve small sized vessels, sometimes even medium sized vessels, are associated with antineutrophil cytoplasmic antibodies with high risk of developing glomerulonephritis and respond well to immunosuppresion with cyclophosphamide.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎是罕见疾病，每年每百万居民中平均有30例新发病例。其主要特征是系统性受累伴血管壁坏死（组织学改变显示中膜坏死及外膜和内膜炎症）。在某些类型中，可发现血管周围有肉芽肿。ANCA相关性血管炎包括肉芽肿性多血管炎（GPA，既往称为韦格纳肉芽肿）、显微镜下多血管炎（MPA）和嗜酸性肉芽肿性多血管炎（EGPA，既往称为变应性肉芽肿性血管炎）。现在，荣誉性的人名命名法正转变为基于疾病描述或病因的命名法。ANCA相关性血管炎是一组独特的血管炎，因为它们主要累及小血管，有时甚至累及中等大小血管，与抗中性粒细胞胞浆抗体相关，发生肾小球肾炎的风险高，且对环磷酰胺免疫抑制治疗反应良好。
