ArticleRMD open2022
Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients.
Article in RMD open, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers, 3 of them syntheses that pooled it.
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Who cites it
39 citing papers in PubMed, 3 syntheses or guidelines pooled it, 82 citations in OpenAlex.
- Diagnosis and treatment strategies for airway involvement in relapsing polychondritis: a comprehensive review.Frontiers in immunology · 2026Pooled it
- Clinical features in VEXAS syndrome: a systematic review.Rheumatology (Oxford, England) · 2025Pooled it
- The role ofInternal and emergency medicine · 2024Pooled it
- Atypical Presentation of VEXAS Syndrome Amid Coexisting Comorbidities: Diagnosis by High Depth Targeted Sequencing.International journal of rheumatic diseases · 2026Article
- VEXAS syndrome unmasked from relapsing polychondritis and infection mimicry: a case-based review.Rheumatology international · 2026Review
- Exploring glucocorticoid dose-response patterns in VEXAS syndrome: a pilot retrospective study.Rheumatology international · 2026Review
- VEXAS syndrome: a comprehensive clinicopathologic and genetic analysis of a predominantly Indian cohort.Journal of hematopathology · 2026Article
- Establishing a consensus definition of VEXAS flare for clinical research.Rheumatology (Oxford, England) · 2026Article
- Disease Trajectories and Glucocorticoid Exposure in VEXAS Syndrome Treated with Cytokine-Directed Therapies.Annals of the rheumatic diseases · 2026Article
- Ophthalmic involvement in VEXAS syndrome and its influence on mortality: insights from the international AIDA network registry.Frontiers in immunology · 2026Article
- The First Reported Case of VEXAS Syndrome in Lebanon: Efficacy of Azacitidine as a Therapeutic Option-Case Report.Case reports in hematology · 2026Article
- What should neurologists expect to observe in relapsing polychondritis and VEXAS?Frontiers in immunology · 2026Review
- Diagnostic and Monitoring Strategies for VEXAS Syndrome: Evaluating Sanger Sequencing, NGS, and the SWIM-Score.Journal of clinical immunology · 2025Article
- Exploring patient-reported outcomes and morbidity burden of patients with VEXAS syndrome: a scoping review.EClinicalMedicine · 2025Review
- Arterial and venous thrombosis in systemic and monogenic vasculitis.Nature reviews. Rheumatology · 2025Review
- Mechanisms of hematopoietic clonal dominance in VEXAS syndrome.Nature medicine · 2025Article
- Laryngeal involvement in relapsing polychondritis: clinical and CT findings in 173 patients.RMD open · 2025Article
- Novel use of Siltuximab in a patient with VEXAS Syndrome.Annals of hematology · 2025Article
- Relapsing polychondritis: tracheobronchial involvement and differential diagnoses.Journal of thoracic disease · 2025Review
- Relapsing Polychondritis With Palmoplantar Pustulosis: A Case Report.Clinical medicine insights. Arthritis and musculoskeletal disorders · 2025Article
Corrections and comments
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Authors and funding
29 authors at 20 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveA new adult-onset autoinflammatory syndrome has been described, named VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic). We aimed to compare the clinical characteristics, the laboratory features and the outcomes between idiopathic-relapsing polychondritis (I-RP) and VEXAS-relapsing polychondritis (VEXAS-RP).
methodsPatients from French retrospective multicentre cohort of RP were separated into two groups: a VEXAS-RP and an I-RP.
resultsCompared with patients with I-RP (n=40), patients with VEXAS-RP (n=55) were men (96% vs 30%, p<0.001) and were older at diagnosis (66 vs 44 years, p<0.001). They had a greater prevalence of fever (60% vs 10%, p<0.001), of skin lesions (82% vs 20%, p<0.001), of ocular involvement (57% vs 28%, p=0.01), of pulmonary infiltrates (46% vs 0%, p<0.001), of heart involvement (11% vs 0%, p=0.0336) and with higher median C-reactive protein levels (64 mg/L vs 10 mg/L, p<0.001). Seventy-five per cent of the patients with VEXAS-RP had myelodysplastic syndrome (MDS) versus none in I-RP group. The glucocorticoids use, and the number of steroid sparing agents were similar in both groups, but patients with VEXAS-RP had more frequent refractory disease (remission obtained in 27% vs 90%, p<0001). VEXAS-RP was associated with higher risk of death: six patients (11%) died in the VEXAS-RP group after a median follow-up of 37 months and none in the I-RP group after a median follow-up of 92 months (p<0.05).
conclusionWe report the largest cohort of VEXAS-RP, characterised by high prevalence of male sex, fever, skin lesion, ocular involvement, pulmonary infiltration, heart involvement, older age and MDS association.
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