Department of Medicine/Division of Rheumatology, Hospital for Special Surgery, New York, NY 10021; email:
Annu Rev Med. 2015;66:227-40. doi: 10.1146/annurev-med-011514-023051. Epub 2014 Oct 17.
The ANCA-associated vasculitides, granulomatosis with polyangiitis (GPA, formerly Wegener's), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss), are a group of multisystem autoimmune diseases characterized by necrotizing small- to medium-vessel vasculitis and the presence of anti-neutrophil cytoplasmic antibodies. Current therapeutic strategies consist of glucocorticoids in conjunction with either conventional or biologic agents for both induction of remission and remission maintenance. Treatment goals include reducing toxicity of induction therapy, preventing disease relapse, and limiting overall accrual of both disease-related damage and treatment-related morbidity. Future research directions include investigation of the optimal duration and frequency of maintenance therapy as well as development of targeted therapeutic agents, which is enhanced by emerging insights into disease pathogenesis.
抗中性粒细胞胞质抗体相关性血管炎，包括肉芽肿性多血管炎（GPA，旧称韦格纳氏肉芽肿）、显微镜下多血管炎（MPA）、嗜酸性肉芽肿性多血管炎（EGPA，旧称变应性肉芽肿性血管炎），是一组以坏死性小至中等血管炎和抗中性粒细胞胞质抗体为特征的多系统自身免疫性疾病。目前的治疗策略包括糖皮质激素联合传统或生物制剂用于诱导缓解和缓解维持。治疗目标包括降低诱导治疗的毒性、预防疾病复发、以及限制疾病相关损害和治疗相关发病率的总累积。未来的研究方向包括探索维持治疗的最佳持续时间和频率，以及开发靶向治疗药物，这得益于对疾病发病机制的新认识。
