University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnet and ERN-RITA Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital, ASL Città di Torino and University of Torino, Turin, Italy.
Rheumatology Unit, Department of Medicine DIMED, University of Padova, Padova, Italy.
Autoimmun Rev. 2024 Jan;23(1):103438. doi: 10.1016/j.autrev.2023.103438. Epub 2023 Aug 30.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) encompasses a group of rare, multisystem autoimmune disorders characterised by the occurrence of inflammation and damage to small blood vessels, leading to a wide range of clinical manifestations. They include granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Outcomes for patients with MPA and GPA have been transformed over recent years. However, the establishment of effective maintenance therapy aiming to balance the risks of disease relapse with those related to prolonged immunosuppression has become a clinical priority. This review aims to explore two differing perspectives on this unsolved problem. Pros and Cons of the following approaches will be discussed: "Biomarker-guided personalised approach on top of generic maintenance strategy guidelines" or "ANCA specificity-related personalised maintenance treatment after intensive B-cell depletion"?
抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）包括一组罕见的多系统自身免疫性疾病，其特征是小血管发生炎症和损伤，导致广泛的临床表现。它们包括肉芽肿性多血管炎（GPA）和显微镜下多血管炎（MPA）。近年来，MPA 和 GPA 患者的预后已经发生了转变。然而，建立有效的维持治疗方法，旨在平衡疾病复发的风险与长期免疫抑制相关的风险，已成为临床的优先事项。本综述旨在探讨这一未解决问题的两种不同观点。将讨论以下方法的优缺点：“在通用维持策略指南基础上的基于生物标志物的个体化方法”或“强化 B 细胞耗竭后基于 ANCA 特异性的个体化维持治疗”？
