1. Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients
- Author
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Vincent Jachiet, Benjamin Terrier, Laurence Bouillet, Claire de Moreuil, Joris Galland, Mathilde Devaux, Mathieu Gerfaud-Valentin, Alexandre Maria, Jean Schmidt, Sylvain Audia, Guillaume Denis, Mohamed-Yacine Khitri, Alexis F Guedon, Maelle le Besnerais, Jean Sebastien Allain, Vincent Grobost, Olivier Kosmider, Anael Dumont, Benjamin Subran, Paola Marianetti-Guingel, Sylvain Palat, Marielle Roux-Sauvat, and Pierre Hirsch
- Subjects
Medicine - Abstract
Objective A 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).Methods Patients from French retrospective multicentre cohort of RP were separated into two groups: a VEXAS-RP and an I-RP.Results Compared with patients with I-RP (n=40), patients with VEXAS-RP (n=55) were men (96% vs 30%, p
- Published
- 2022
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