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Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients

Title: Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients
Authors: 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
Source: RMD Open, Vol 8, Iss 2 (2022)
Publisher Information: BMJ Publishing Group
Publication Year: 2022
Collection: Directory of Open Access Journals: DOAJ Articles
Subject Terms: Medicine
Description: 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
Document Type: article in journal/newspaper
Language: English
Relation: https://rmdopen.bmj.com/content/8/2/e002255.full; https://doaj.org/toc/2056-5933; https://doaj.org/article/a959da3d9d8e409592a5aec954add3f0
DOI: 10.1136/rmdopen-2022-002255
Availability: https://doi.org/10.1136/rmdopen-2022-002255; https://doaj.org/article/a959da3d9d8e409592a5aec954add3f0
Accession Number: edsbas.CEE5D59B
Database: BASE