Department of Rheumatology and Clinical Immunology, University of Lübeck, Germany.
Department of Nephrology, Asklepios Klinikum Barmbek, Hamburg; and III Department of Medicine, Division of Rheumatology and Systemic Inflammatory Diseases, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Clin Exp Rheumatol. 2024 Apr;42(4):852-858. doi: 10.55563/clinexprheumatol/suxkyq. Epub 2024 Apr 12.
Prospective long-term observational data on the disease course of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were missing in Germany to date. Therefore, the Joint Vasculitis Registry in German-speaking countries (GeVas) has been established to follow the course of patients with AAV. The aim of this study is to present baseline data of patients with newly diagnosed and relapsing AAV enrolled in the GeVas registry.
GeVas is a prospective, web-based, multicentre, clinician-driven registry for the documentation of organ manifestations, damage, long-term outcomes, and therapy regimens in various types of vasculitis. Recruitment started in June 2019.
Between June 2019 and October 2022, 266 patients with AAV were included in the GeVas registry: 173 (65%) with new-onset and 93 (35%) with relapsing AAV. One hundred and sixty-two (61%) patients were classified as granulomatosis with polyangiitis (GPA), 66 (25%) as microscopic polyangiitis (MPA), 36 (13%) as eosinophilic granulomatosis with polyangiitis (EGPA), and 2 (1%) as renal limited AAV. The median age was 59 years (51-70 years, IQR), 130 (51%) patients were female. Most patients were ANCA positive (177; 67%) and affected by general symptoms, pulmonary, ear nose throat (ENT), renal and neurological involvement. For induction of remission, the majority of patients received glucocorticoids (247, 93%) in combination with either rituximab (118, 45%) or cyclophosphamide (112, 42%).
Demographic characteristics are comparable to those in other European countries. Differences were found regarding ANCA status, frequencies of organ manifestations, and therapeutic regimens. The GeVas registry will allow longitudinal observations and prospective outcome measures in AAV.
德国目前缺乏抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）疾病过程的前瞻性长期观察数据。因此，建立了德语国家联合血管炎登记处（GeVas）以跟踪 AAV 患者的病情。本研究旨在介绍新诊断和复发的 AAV 患者在 GeVas 登记处的基线数据。
GeVas 是一个前瞻性的、基于网络的、多中心的、由临床医生驱动的登记处，用于记录各种类型血管炎的器官表现、损伤、长期结果和治疗方案。招募工作于 2019 年 6 月开始。
2019 年 6 月至 2022 年 10 月期间，GeVas 登记处共纳入 266 例 AAV 患者：173 例（65%）为新发病例，93 例（35%）为复发病例。162 例（61%）患者被诊断为肉芽肿性多血管炎（GPA），66 例（25%）为显微镜下多血管炎（MPA），36 例（13%）为嗜酸性肉芽肿性多血管炎（EGPA），2 例（1%）为肾局限性 AAV。中位年龄为 59 岁（51-70 岁，IQR），130 例（51%）为女性。大多数患者为 ANCA 阳性（177 例；67%），并伴有全身症状、肺部、耳鼻喉（ENT）、肾脏和神经系统受累。为了诱导缓解，大多数患者接受了糖皮质激素（247 例，93%）联合利妥昔单抗（118 例，45%）或环磷酰胺（112 例，42%）。
人口统计学特征与其他欧洲国家相似。在 ANCA 状态、器官表现频率和治疗方案方面存在差异。GeVas 登记处将允许对 AAV 进行纵向观察和前瞻性结局测量。
