对ANCA相关性血管炎的认识和争议

Antineutrophil cytoplasm antibody-associated vasculitis:understandings and controversies

中性粒细胞质抗体(ANCA)相关性血管炎(AAV)是一组临床表现为ANCA阳性并且累及多系统的寡免疫复合物型小血管炎。AAV在老年人群中更为常见,病因尚不清楚,可导致器官和血管的局灶坏死。若累及肺脏可导致肺泡出血,肾脏受累则可表现为新月体性肾炎和急性肾衰竭,皮肤表现为紫癜性皮疹和溃疡。临床常使用糖皮质激素及免疫抑制剂治疗,部分患者需要血浆置换。笔者对AAV的病因、发病机制和分类进行探讨,阐述了一些不同的认识和争议。

Antineutrophil cytoplasm antibody（ANCA）-associated vasculitis（AAV） is a group of vasculitides characterized by a pauci-immune small-to-medium sized vessel vasculitis and the presence of circulating ANCA.AAV is increasingly prevalent in the elderly population and the causes of AAV are still not known.These diseases can cause the focal necrotizing lesions which affect vessels and organs.They may cause alveolar hemorrhage in the lungs,crescentic glomerulonephritis with acute renal failure in the kidneys,and purpuric rash and ulcerations in the skin.Treatment usually includes corticosteroids,immunosuppresive therapy and in some cases plasmapheresis.In this paper,the authors discussed some different viewpoints and controversies about the etiology,pathogenesis and classification of AAV.