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[我如何治疗ANCA血管炎与脏参与]

Maxime Crucil¹, Céline Regnier², Christian Von Frenckell²

¹Service de Néphrologie, CHU Liège, Belgique.

Revue medicale de Liege

|September 11, 2025

概括

与ANCA相关的血管炎可能会导致脏损伤. 新的指导方针建议早期进行脏活检和阿瓦科潘等治疗,以控制这种罕见疾病并预防功能衰竭.

科学领域:

腎臟病學 (nephrology) 是一種醫學專業.
类风湿病学类风湿病学
免疫学免疫学免疫学

背景情况:

与ANCA相关的血管炎 (AAV),包括与多炎 (GPA) 和显微多炎 (MPA) 相关的颗粒性血管炎,是罕见的全身性疾病,其特征是小血管的瘤性炎症.
脏干扰是常见的并发症,通常表现为急性损伤与出血和蛋白尿.

研究的目的:

总结目前对ANCA相关血管炎的理解和管理策略,重点关注脏参与.
突出KDIGO 2024指南中关于AAV的诊断和治疗的建议.

主要方法:

对诊断标准的审查,包括血清学测试 (PR3-ANCA,MPO-ANCA) 和组织学.
对诱导和维持阶段的治疗方法的分析,包括皮质类固醇,环胺,修复剂和阿瓦科潘.
整合KDIGO 2024指导方针的建议.

主要成果:

诊断依赖于特定的自身抗体和脏活检结果,经验证的得分预测进展到末期脏疾病.
诱导疗法通常涉及皮质类固醇和免疫抑制剂 (cyclophosphamide或rituximab);维持疗法旨在防止复发,而rituximab显示出优于azathioprine的优势.
阿瓦科潘是一种C5a受体抑制剂,作为一种有希望的选择,可以在诱导期间减少对皮质类固醇的依赖.

结论:

该KDIGO 2024指南强调早期脏活检和标准化免疫抑制方案用于AAV管理.
像阿瓦科潘这样的创新疗法提供了新的视角,提高了治疗疗效,并可能减少与皮质类固醇相关的副作用.
有效管理AAV至关重要,以防止不可逆转的损伤和改善患者的结果.

关键词:

与ANCA相关的血管炎急性功能衰竭急性功能衰竭是什么这是Avacopan.在KDIGO 2024年脏活检 - 脏活检

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