Ahn Sung Soo, Lee Sang-Won
Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin, Korea.
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
J Rheum Dis. 2023 Apr 1;30(2):72-87. doi: 10.4078/jrd.2022.0002. Epub 2023 Mar 17.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune rheumatic disease consisting of three discrete diagnoses of microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis. Among diseases treated in a rheumatology department, AAV has poor clinical outcomes, with high rates of mortality and progression to end-stage renal disease and frequent disease relapse. Due to the frequent negative patient outcomes, optimal therapeutic strategies are essential in the management of AAV. In the present review, four guidelines for management of AAV are summarized: British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guideline for the management of adults with AAV; European League Against Rheumatism (EULAR)/European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) recommendation for the management of AAV; 2021 American College of Rheumatology (ACR)/Vasculitis Foundation Guideline for the Management of AAV; Kidney Disease: Improving Global Outcome (KDIGO) 2021 Clinical Practice Guideline for the Management of Glomerular Diseases, which will aid in clinicians' medical decisions. Finally, the summary of the 2022 Update of the EULAR Recommendations on the Management of AAV, presented in the EULAR Congress 2022 is also introduced.
抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)是一种自身免疫性风湿性疾病,由显微镜下多血管炎、肉芽肿性多血管炎和嗜酸性肉芽肿性多血管炎三种不同的诊断组成。在风湿科治疗的疾病中,AAV的临床预后较差,死亡率高,易进展为终末期肾病,且疾病复发频繁。由于患者预后往往不佳,因此最佳治疗策略对于AAV的管理至关重要。在本综述中,总结了四项AAV管理指南:英国风湿病学会(BSR)和英国风湿病健康专业人员(BHPR)关于成人AAV管理的指南;欧洲抗风湿病联盟(EULAR)/欧洲肾脏协会 - 欧洲透析和移植协会(ERA - EDTA)关于AAV管理的建议;2021年美国风湿病学会(ACR)/血管炎基金会AAV管理指南;肾脏病:改善全球预后(KDIGO)2021年肾小球疾病管理临床实践指南,这些将有助于临床医生做出医疗决策。最后,还介绍了在2022年EULAR大会上发布的2022年EULAR AAV管理建议更新摘要。