Gattu Rishma, Demory Beckler Michelle, Kesselman Marc M
Osteopathic Medicine, Nova Southeastern University Dr. Kiran C. Patel College of Osteopathic Medicine, Fort Lauderdale, USA.
Microbiology and Immunology, Nova Southeastern University Dr. Kiran C. Patel College of Allopathic Medicine, Fort Lauderdale, USA.
Cureus. 2024 Aug 18;16(8):e67161. doi: 10.7759/cureus.67161. eCollection 2024 Aug.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises a spectrum of autoimmune diseases, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Studies have shown that avacopan and mepolizumab are promising therapeutics for partial or complete replacement of glucocorticoids (GC), with sustained remission while completely weaning off GC. Avacopan inhibits C5aR in the complement pathway, preventing neutrophil migration, while mepolizumab targets IL-5R, reducing eosinophil activity. Additionally, complement inhibition has not only contributed to the recovery of renal function and alleviation of physical symptoms but has also enhanced patients' overall quality of life and mental well-being. This systematic review explores the pathogenesis of AAV, traditional treatments, and the potential of emerging complement and interleukin antagonist therapies such as avacopan and mepolizumab in revolutionizing AAV management.
抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)包括一系列自身免疫性疾病,包括肉芽肿性多血管炎(GPA)、显微镜下多血管炎(MPA)和嗜酸性肉芽肿性多血管炎(EGPA)。研究表明,阿伐库潘和美泊利单抗有望部分或完全替代糖皮质激素(GC),在完全停用GC的情况下实现持续缓解。阿伐库潘抑制补体途径中的C5aR,阻止中性粒细胞迁移,而美泊利单抗靶向IL-5R,降低嗜酸性粒细胞活性。此外,补体抑制不仅有助于肾功能的恢复和身体症状的缓解,还提高了患者的整体生活质量和心理健康。本系统评价探讨了AAV的发病机制、传统治疗方法,以及阿伐库潘和美泊利单抗等新兴补体和白细胞介素拮抗剂疗法在彻底改变AAV治疗方面的潜力。