嗜酸细胞肉芽肿性血管炎抗中性粒细胞胞浆抗体阳性与阴性患者临床特征

Clinical features of antineutrophil cytoplasmic antibodies positive and negative eosinophilic granulomatosis with polyangiitis

嗜酸细胞肉芽肿性血管炎(EGPA)是以外周血和受累脏器组织中嗜酸性粒细胞增高并伴有坏死性肉芽肿为特征的系统性血管炎,依据血清抗中性粒细胞胞浆抗体(ANCA)检测结果可分为ANCA阳性及ANCA阴性表型,ANCA阳性患者更易出现神经系统、肾脏、皮肤紫癜和肺泡出血;心脏和肺受累在ANCA阴性患者中更常见。本文就ANCA阳性与阴性患者的临床表现及受累脏器特征进行文献复习,并综述如下。

Eosinophilic granulomatosis with polyangiitis （EGPA） is a rare systemic vasculitis characterized by eosinophil-rich and necrotizing granulomatous inflammation in the peripheral blood and involved organs.According to whether antineutrophil cytoplasmic auto-antibodies （ANCA） can be detected in the serum of the patients or not, EGPA is assigned into ANCA-positive or-negative phenotypes.The ANCA-positive patients are prone to purpura, glomerulonephritis, peripheral neuropathy, and alveolar hemorrhage.Myocardial and lung involvement are more common in those with ANCA-negative.In this article, we reviewed the literatures concerning the differences in the clinical manifestations and involved organs between the ANCA-positive and-negative EGPA, and summarized their clinical characteristics.