Search

Background: 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., Purpose: 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., Methodology: 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., Results: 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., Conclusion: 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., Primary Funding Source: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors., Competing Interests: Declaration of Competing Interest Laurent ARNAUD is a consultant for Alexion, Amgen, Astra-Zeneca, Abbvie, Biogen, BMS, Boehringer-Ingelheim, Cêmka, GSK, Janssen, Kezar, LFB, Lilly, Menarini France, Medac, Novartis, Oséus, Pfizer, Roche-Chugaï, Sêmeia, UCB., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Purpose of Review: Rheumatoid vasculitis is the most serious extra-articular complication of rheumatoid arthritis, with high morbidity and mortality noted in multiple prior reports. Recent studies have expanded our understanding of this entity in the era of modern immunosuppressive therapy. New clinical predictors and possible protective factors have also been identified., Recent Findings: This review provides an update on the epidemiology, clinical correlates, predictors, therapy and outcomes of rheumatoid vasculitis over the past decade. During this time, there has been increasing use of the treat-to-target management practices and incorporation of biologic response modifiers that have revolutionized rheumatoid arthritis treatment with better disease control and overall improved outcomes. The incidence of rheumatoid vasculitis has declined significantly in the past several decades, but morbidity and mortality continue to remain high, despite aggressive treatment with cyclophosphamide or biologic agents. Hydroxychloroquine and low-dose aspirin may have a protective role. There is ongoing debate about the role of newer biological therapies in prevention, treatment or even as a trigger for rheumatoid vasculitis., Summary: Rheumatoid vasculitis remains a rare yet challenging extra-articular manifestation of rheumatoid arthritis with high morbidity and mortality, despite aggressive use of disease-modifying therapy.