Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients
| Autor: | 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, Pierre Hirsch |
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| Jazyk: | angličtina |
| Rok vydání: | 2022 |
| Předmět: | |
| Zdroj: | RMD Open, Vol 8, Iss 2 (2022) |
| Druh dokumentu: | article |
| ISSN: | 2022-0022 2056-5933 |
| DOI: | 10.1136/rmdopen-2022-002255 |
| Popis: | 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 |
| Databáze: | Directory of Open Access Journals |
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