嗜酸性肉芽肿性血管炎的诊治进展

Progress in the Diagnosis and Treatment of Eosinophilic Granulomatosis with Polyangiitis

嗜酸性肉芽肿性血管炎(EGPA)是一种系统性血管炎性疾病,以坏死性血管炎、组织器官嗜酸粒细胞(EOS)浸润和血管外肉芽肿形成为主要特征。其发病主要与免疫遗传因素和环境刺激有关,而EOS组织器官浸润和抗中性粒细胞胞质抗体介导的血管内皮损伤被认为是最重要的发病机制,EGPA临床表现缺乏特异性,诊断多采用美国风湿病协会制定的临床诊断标准,EGPA治疗需根据患者的预后不良因素制订治疗方案,大剂量糖皮质激素联合环磷酰胺仍然是有预后不良因素患者的首选治疗方案,而生物制剂在诱导疾病缓解方面也取得可喜成果;儿童EGPA病例相比成人更为罕见并有其自身的特点。

Eosinophilic granulomatosis with polyangiitis（EGPA） is a systemic vascular inflammatory disease,characterized by necrotizing vasculitis,extravascular granulomas and tissue eosinophilia（EOS）. Different environmental factors have been suggested as potential triggers of EGPA,and immunogenetic factors may confer susceptibility to EGPA. ANCA-induced endothelial damage and EOS infiltration are probably the most important mechanisms of disease pathogenesis. The American College of Rheumatology criteria is the most widely used set of criteria to diagnose EGPA,and the treatment of patients with EGPA should be tailored to individual patients according to the presence of poor prognostic factors. The gold standard for the treatment of the cases with poor-prognosis factors is a combination of cyclophosphamide and high-dose corticosteroids,while biological agent has shown promising results for remission induction. Childhood EGPA cases are rare compared to adults,and have their own characteristics.