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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.)

Objectives: To document the current training, implementation and role of modern musculoskeletal imaging techniques: ultrasound, magnetic resonance imaging, computed tomography and positron emission tomography, among rheumatologists in the member countries of the EULAR., Methods: English-language questionnaires for each imaging modality developed by a EULAR task force were sent out to national and international scientific societies as well as imaging experts in the given modalities involved in research and/or training. The surveys were distributed via an online survey tool (SurveyMonkey). Simple descriptive and summary statistics were calculated from the responses., Results: More than 90% of ultrasound (US) experts reported the availability of a US unit in their department. Suspicion of rheumatoid arthritis and spondyloarthritides were the main clinical indications for performing US for diagnostic purposes. Suspicion of sacroiliitis and degenerative spine disease were the most common indications to perform magnetic resonance imaging (MRI) or computed tomography (CT) for diagnostic purposes, while positron emission tomography was mainly performed to diagnose large vessel vasculitis and to investigate fever of unknown origin. The reported percentage of rheumatologists performing US was highly variable, ranging from more than 80% in 6% of countries to less than 10% in 15% of countries. The majority of experts (77%) reported that their national rheumatology societies organise musculoskeletal US courses, while courses in MRI or CT organised by the national rheumatology societies were less commonly reported (29% and 8%, respectively)., Conclusions: Rheumatologists in Europe utilise modern imaging techniques; however, access to the techniques and training offered is varied., Competing Interests: Competing interests: None declared.

Purpose of Review: Rheumatoid vasculitis (RV) is an unusual complication of long-standing rheumatoid arthritis, which is characterized by the development of necrotizing or leukocytoclastic vasculitis involving small or medium-sized vessels. In this review, we aim to provide an update on the epidemiology, pathogenesis, clinical presentation, and management of this challenging extra-articular manifestation., Recent Findings: RV is heterogenous in its clinical presentation depending on the organ and size of blood vessels involved. The most common organs involved are the skin and peripheral nerve. Based on recent population studies, the incidence has significantly decreased with early recognition and the advent of immunosuppressive drugs and biologics; however, the mortality rates remain high. RV remains a serious extra-articular manifestation of RA that needs to be promptly recognized and treated. No consensus is available on treatment, given the ongoing debate of whether the biologics can trigger or treat RV.

Vasculitis in rheumatoid arthritis (rheumatoid vasculitis, RV) has a heterogeneous clinical presentation that includes skin disorders, neuropathy, eye symptoms and systemic inflammation. Rheumatoid vasculitis is an unusual complication of longstanding, severe rheumatoid arthritis (RA). While RA affects the body's joints, vasculitis is a condition in which blood vessels become inflamed. Rheumatoid vasculitis occurs in approximately 2 to 5% of patients who have RA. The blood vessels most often involved are arteries that bring blood to the skin, nerves, and internal organs. Veins can also be involved. Rheumatoid vasculitis is skin condition that is a typical feature of RA, presenting as peripheral vascular lesions that are localized (purpura, cutaneous ulceration, and gangrene of the distal parts of the extremities). The cause of RV is unknown, but given the prominence of immune components and the pathologic changes in involved blood vessels, an autoimmune process is suggested. Compared to other forms of vasculitis, there has been relativejy little research in recent years on the specific entity of RV. There is some evidence that the incidence of RV has decreased over the past several decades, perhaps because of a better treatment of the underlying RA. In the present review, we discuss the clinical features, laboratory tests, the pathogenesis of RV.

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.

Objective: The aim of this study was to determine the clinical correlates and predictors of rheumatoid vasculitis (RV)., Methods: A retrospective cohort of patients with RV evaluated at a tertiary referral centre between 1 January 2000 and 1 January 2010 was identified. RV cases were compared in a 1:2 ratio to controls (RA without vasculitis) to identify risk factors for developing RV., Results: Eighty-six RV cases (58% women, 88% white) were identified. Histopathological confirmation was available for 58% of patients. Cutaneous vasculitis was the most common presentation, followed by vasculitic neuropathy. The median age at presentation was 63 years and the median duration of RA was 10.8 years. One third were current smokers. The majority were seropositive and had elevated inflammatory markers. Treatment was with a range of immunomodulating agents. At 6 months, 38% of patients achieved complete remission, 52% had partial improvement and 10% noted no clinical improvement. Thirty-six per cent relapsed by 5 years and 26% died. After adjusting for age and disease duration, current smoking at RA diagnosis [odds ratio (OR) 1.98], coexistent peripheral vascular disease (OR 3.98), cerebrovascular disease (OR 6.48), severe RA (OR 2.02) (characterized by radiographic erosions, nodulosis on clinical examination or requirement of joint surgery) and the use of biologics (OR 2.80) were found to increase the odds for developing RV; the use of HCQ (OR 0.54, CI 0.31, 0.94) and low-dose aspirin (OR 0.42, CI 0.21, 0.85) was associated with decreased odds for developing RV., Conclusion: This largest single-centre series of patients with RV suggests that even in recent years, RV remains a serious complication of RA and is associated with significant mortality.

Objectives: Systemic rheumatoid vasculitis (SRV) is a rare but potentially serious systemic disease manifestation of rheumatoid arthritis (RA) characterized by the development of necrotizing vasculitis. The incidence of SRV appears to be decreasing possibly reflecting progress in RA treatment. The aims of this study were to review the clinical manifestations of SRV in a stable well-defined population during 2001-10 and to compare with our previous cohort (1988-2000) and also a cohort from 1975 to 1981., Methods: Using Norfolk Vasculitis Register, a prospective register of patients with systemic vasculitis since 1988, all patients with a diagnosis of SRV from 1 January 2001 until 31 December 2010 were identified. SRV was defined according to the Scott and Bacon criteria (1984). Clinical features were obtained by retrospective case note review., Results: Eighteen patients with SRV were identified (10 male), median age at diagnosis was 72 years and average disease duration 15.6 years. The average annual incidence for 2001-10 was 3.9 per million. One-year mortality was 12% and 5-year mortality 60%. The clinical manifestations were similar apart from systemic and cutaneous features which were more common in the earlier cohorts., Conclusion: The incidence of SRV has declined significantly in the last 40 years; but the clinical manifestations remain similar. Systemic symptoms, and cutaneous manifestations such as infarcts and nodules, are slightly less common in the recent cohort. Despite modern immunosuppressive therapy the prognosis remains poor.

Objective: To assess incidence and mortality effects of extraarticular rheumatoid arthritis (ExRA) in patients with incident RA in 1995-2007 compared to 1985-1994, in Olmsted County, Minnesota, USA., Methods: Data on incident ExRA were abstracted from medical records of patients with RA - Olmsted County residents who first met the 1987 American College of Rheumatology criteria for RA between January 1, 1995, and December 31, 2007. Patients were followed until death, migration from Olmsted County, or December 31, 2008. ExRA were classified using the predefined criteria and compared to the corresponding 1985-1994 inception RA cohort (n = 147)., Results: The 1995-2007 cohort included 463 patients with RA followed for a mean of 6.3 years; mean age was 55.6 years, 69% were women, 67% were positive for rheumatoid factor (RF). The 10-year cumulative incidence of any ExRA (50.1%) and severe ExRA (6.7%) in the 1995-2007 cohort was similar to the 1985-1994 cohort (46.2% and 9.7%, respectively). The 10-year cumulative incidence of vasculitis, but not other features of ExRA, was significantly lower in the 1995-2007 cohort (0.6%) compared to the 1985-1994 cohort (3.6%). RF positivity, erosions/destructive changes, and use of methotrexate, other disease-modifying antirheumatic drugs and systemic corticosteroids were significantly associated with ExRA in the 1995-2007 cohort. ExRA was associated with mortality risk (HR 2.1, 95% CI 1.2, 3.7) in the 1995-2007 cohort. The decrease in mortality following ExRA in the 1995-2007 cohort versus the 1985-1994 cohort did not reach statistical significance (HR 0.6, 95% CI 0.3, 1.2, p = 0.16)., Conclusion: ExRA remains a common complication associated with increased mortality in RA. The occurrence of vasculitis appears to be decreasing in recent years.