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中华肾病研究电子杂志 ›› 2024, Vol. 13 ›› Issue (06) : 345 -351. doi: 10.3877/cma.j.issn.2095-3216.2024.06.008

综述

抗中性粒细胞胞质抗体相关性血管炎治疗研究进展
帖璇1,2, 苏晓乐1,2,(), 王利华1,2,()   
  1. 1.030001 太原,山西医科大学
    2.030000 太原,山西医科大学第二医院肾内科
  • 收稿日期:2023-12-01 出版日期:2024-12-28
  • 通信作者: 苏晓乐, 王利华

Progress of research on the treatment of anti-neutrophil cytoplasmic antibody-associated vasculitis

Xuan Tie1,2, Xiaole Su1,2,(), Lihua Wang1,2,()   

  1. 1.Shanxi Medical University, Taiyuan 030001
    2.Department of Nephrology, Second Hospital of Shanxi Medical University, Taiyuan 030000; Shanxi Province, China
  • Received:2023-12-01 Published:2024-12-28
  • Corresponding author: Xiaole Su, Lihua Wang
引用本文:

帖璇, 苏晓乐, 王利华. 抗中性粒细胞胞质抗体相关性血管炎治疗研究进展[J/OL]. 中华肾病研究电子杂志, 2024, 13(06): 345-351.

Xuan Tie, Xiaole Su, Lihua Wang. Progress of research on the treatment of anti-neutrophil cytoplasmic antibody-associated vasculitis[J/OL]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2024, 13(06): 345-351.

抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)是一组以小血管炎为特征的系统性自身免疫性疾病,其病理机制复杂且具有较高的复发率。 近年来,随着对AAV 病理生理机制的深入理解,治疗策略取得了显著进展。 本文就AAV 诱导缓解治疗、维持缓解治疗及复发病例管理策略的研究进展进行综述,旨在为优化治疗方案、开发新型药物提供思路。

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of systemic autoimmune diseases characterized by small vessel vasculitis, with a complex pathological mechanism and a high recurrence rate. In recent years, significant progress has been made in treatment strategies with the deepening understanding of the pathophysiological mechanisms of AAV. This article reviewed the progress of research on AAV treatment, including remission-induction therapy, remissionmaintenance therapy, and management strategies for recurrent cases, aiming to provide ideas for optimizing treatment plans and developing new drugs.

表1 PEXVAS 研究和LoVAS 研究内容比较
表2 PEXVAS 研究和LoVAS 研究减量糖皮质激素方案的比较
表3 抗中性粒细胞胞浆抗体相关性血管炎复发的危险因素[25]
表4 抗中性粒细胞胞浆抗体相关性血管炎主要药物治疗汇总表
图1 用于治疗肉芽肿性多血管炎或显微镜下多血管炎的流程图[12] 注:AZA:azathioprine,硫唑嘌呤;CTX:cyclophosphamide,环磷酰胺;GC:glucocorticoid,糖皮质激素;GPA:granulomatosis with polyangiitis, 肉芽肿性多血管炎;MMF:mycophenolate mofetil,吗替麦考酚酯;MPA:microscopic polyangiitis,显微镜下多血管炎;MTX:methotrexate,甲氨蝶呤;PLEX:plasma exchange,血浆置换;RPGN:rapidly progressive glomerulonephritis,快速进行性肾小球肾炎;RTX:rituximab,利妥昔单抗;①虚线表示需要考虑补充的治疗方案;②图中GC 剂量以泼尼松龙为例,目前考虑在无危及器官损害或危及生命的患者中降低起始剂量为0.5 mg/d;③在复发性疾病和有生育能力或既往暴露于CTX 的患者(男性/女性)中,RTX 优于CTX,认为个体累积剂量与并发症风险增加相关;④由于活动性肾小球肾炎导致血清肌酐>300 μmol/L 的患者,考虑到终末期肾病的个体风险和患者偏好,可考虑血浆置换;⑤治疗6 ~12 个月后停用avacopan,暂无超过1 年的avacopan 使用数据,因此不建议长期使用
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