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德语国家联合血管炎登记处(GeVas):195 例 GCA 患者的亚组分析。

The Joint Vasculitis Registry in German-speaking countries (GeVas): subgroup analysis of 195 GCA patients.

机构信息

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.

Department of Rheumatology and Clinical Immunology, University of Lübeck, Germany.

出版信息

Clin Exp Rheumatol. 2024 Apr;42(4):895-904. doi: 10.55563/clinexprheumatol/d3o0gu. Epub 2024 Apr 19.

Abstract

OBJECTIVES

Giant cell arteritis (GCA) is one of the most common forms of vasculitis. There is an abundance of studies which are conducted in a randomised controlled trial setting but limited with respect to cohort size and follow-up time. GeVas is the first large-scale registry for vasculitides in German-speaking countries that enables to evaluate this rare disease. Herein we focus on the subgroup of GCA patients including follow-up data up to one year.

METHODS

GeVas is a prospective, web-based, multicentre registry for the documentation of organ manifestations, outcomes, and therapy regimens in vasculitides. Recruitment started in June 2019. By April 2023, 15 centres were initiated and have started to enrol patients.

RESULTS

After 4 years, 195 GCA-patients were included in the registry, of which 64% were female and 36% were male. The average age was 76 years at the time of recruitment (IQR=69-82). Seventy-nine percent were included in the registry because of a newly diagnosed GCA and 21% because of a relapse. At the first assessment most of the patients (89%) described general symptoms. Thirty-one percent stated ocular symptoms. Cranial symptoms were documented in 78% of the cases. All patients were documented with immunosuppressive treatment at start, of whom 95% received prednisolone, 16% cyclophosphamide, 20% methotrexate, and 48% tocilizumab. After three months 62% and after one year 91% of the patients achieved remission.

CONCLUSIONS

Regarding demographics, clinical manifestations and diagnostics, our study showed a similar composition compared to other studies. However, our data differed in terms of treatment regimens.

摘要

目的

巨细胞动脉炎(GCA)是最常见的血管炎形式之一。有大量的研究是在随机对照试验中进行的,但在队列规模和随访时间方面有限。GeVas 是德语国家首个用于评估这种罕见疾病的大型血管炎登记处。在此,我们重点关注 GCA 患者亚组,包括长达一年的随访数据。

方法

GeVas 是一个前瞻性的、基于网络的、多中心的血管炎器官表现、结局和治疗方案登记处。招募工作于 2019 年 6 月开始。截至 2023 年 4 月,已有 15 个中心启动并开始招募患者。

结果

4 年后,登记处共纳入 195 例 GCA 患者,其中 64%为女性,36%为男性。招募时的平均年龄为 76 岁(IQR=69-82)。79%的患者因新诊断的 GCA 而被纳入登记处,21%的患者因复发而被纳入。首次评估时,大多数患者(89%)描述了全身症状。31%的患者出现眼部症状。78%的病例记录了颅部症状。所有患者在开始时均接受免疫抑制治疗,其中 95%接受泼尼松龙治疗,16%接受环磷酰胺治疗,20%接受甲氨蝶呤治疗,48%接受托珠单抗治疗。三个月后 62%的患者和一年后 91%的患者达到缓解。

结论

就人口统计学、临床表现和诊断而言,我们的研究与其他研究相比表现出相似的构成。然而,我们的数据在治疗方案方面存在差异。

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