Service de rhumatologie, INSERM UMR-S1109, Hôpital de Hautepierre, 1 Avenue Molière BP 83049, 67098 Strasbourg Cedex, France; Centre National de Référence des Maladies Auto-immunes Systémiques Rares Est Sud-Ouest (RESO)-LUPUS, European Reference Networks (ERN) ReCONNET and RITA, France.
Dermatology Clinic, Hôpitaux Universitaires et Université de Strasbourg, 1 Place de l'Hôpital, 67091 Strasbourg Cedex, France.
Autoimmun Rev. 2023 Sep;22(9):103391. doi: 10.1016/j.autrev.2023.103391. Epub 2023 Jul 17.
Significant changes in the epidemiology and natural history of rheumatoid vasculitis (RV) have occurred with the introduction of biological therapies such as TNF inhibitors (TNFi) and rituximab.
This scoping review aims to address the key current challenges and propose updated criteria for RV. This will aid future descriptive observational studies and prospective therapeutic trials.
The MEDLINE database was searched for eligible articles from inception through December 2022. Articles were selected based on language and publication date after 1998, corresponding to the approval of the first TNFi in rheumatic diseases.
Sixty articles were included in the review. The mean incidence of RV has decreased since the approval of biologic therapies in RA, from 9.1 (95% CI: 6.8-12.0) per million between 1988 and 2000 to 3.9 (95% CI: 2.3-6.2) between 2001 and 2010, probably due to significant improvement in RA severity and a decrease in smoking habits. Factors associated with an increased risk of RV include smoking at RA diagnosis, longer disease duration, severe RA, immunopositivity, and male gender (regardless of age). Homozygosity for the HLA-DRB104 shared epitope is linked to RV, while the presence of HLA-C3 is a significant predictor of vasculitis in patients without HLA-DRB104. Cutaneous (65-88%), neurologic (35-63%), and cardiac (33%) manifestations are common in RV, often associated with constitutional symptoms (70%). Histologic findings range from small vessel vasculitis to medium-sized necrotizing arteritis, but definite evidence of vasculitis is not required in the 1984 Scott and Bacon diagnostic criteria. Existing data on RV treatment are retrospective, and no formal published guidelines are currently available.
The understanding of RV pathogenesis has improved since its initial diagnostic criteria, with a wider range of clinical manifestations identified. However, a validated and updated criteria that incorporates these advances is currently lacking, impeding the development of descriptive observational studies and prospective therapeutic trials.
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
随着 TNF 抑制剂（TNFi）和利妥昔单抗等生物疗法的引入，类风湿性血管炎（RV）的流行病学和自然史发生了重大变化。
本范围界定综述旨在解决当前的主要挑战，并提出 RV 的更新标准。这将有助于未来的描述性观察性研究和前瞻性治疗试验。
通过 MEDLINE 数据库搜索自 1998 年以来发表的合格文章，以语言和出版日期为基础进行选择。
综述共纳入 60 篇文章。自生物疗法批准用于治疗类风湿关节炎以来，RV 的平均发病率有所下降，从 1988 年至 2000 年的每百万 9.1 例（95%CI：6.8-12.0）降至 2001 年至 2010 年的每百万 3.9 例（95%CI：2.3-6.2），这可能是由于 RA 严重程度的显著改善和吸烟习惯的减少。与 RV 风险增加相关的因素包括 RA 诊断时吸烟、疾病持续时间较长、严重 RA、免疫阳性和男性（无论年龄大小）。HLA-DRB104 共享表位的纯合性与 RV 相关，而 HLA-C3 的存在是 HLA-DRB104 阴性患者血管炎的重要预测因子。RV 常见的皮肤（65-88%）、神经（35-63%）和心脏（33%）表现，常伴有全身症状（70%）。组织学发现从小血管血管炎到中等大小的坏死性动脉炎不等，但在 1984 年 Scott 和 Bacon 诊断标准中并不需要明确的血管炎证据。目前关于 RV 治疗的数据是回顾性的，尚无正式的发表指南。
自最初的诊断标准以来，对 RV 发病机制的理解有所提高，确定了更广泛的临床表现。然而，目前缺乏一种经过验证和更新的标准，这阻碍了描述性观察性研究和前瞻性治疗试验的发展。
这项研究没有得到公共、商业或非营利部门任何特定资助机构的资金支持。
