Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients
Year: 2022
Type: article
Study design: Observational Study
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<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). CONCLUSION: We 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.
Source: RMD Open
Authors: Mohamed-Yacine Khitri, Alexis F Guedon, Sophie Georgin-Lavialle, Benjamin Terrier, David Saadoun, Julie Seguier, Maelle le Besnerais, Claire De Moreuil, Guillaume Denis, Mathieu Gerfaud-Valentin, Jean Sebastien Allain, Alexandre Maria, Laurence Bouillet, Vincent Grobost, Joris Galland, Olivier Kosmider, Anael Dumont, Mathilde Devaux, Benjamin Subran, Jean Schmidt, Paola Marianetti-Guingel, Sylvain Audia, Sylvain Palat, Marielle Roux-Sauvat, Vincent Jachiet, Pierre Hirsch, Olivier Fain, Arsène Mekinian
Institutions: Assistance Publique – Hôpitaux de Paris, Hôpital Saint-Antoine, Institut Pierre Louis d‘Épidémiologie et de Santé Publique, Hôpital Tenon, Hôpital Cochin, Pitié-Salpêtrière Hospital, Aix-Marseille Université, Hôpital de la Timone, Hôpital Charles-Nicolle, Centre Hospitalier Régional Universitaire de Brest, Centre Hospitalier de Rochefort, Hospices Civils de Lyon, Centre Hospitalier Universitaire de Rennes, Centre Hospitalier Universitaire de Montpellier, Institute for Regenerative Medicine & Biotherapy, Centre Hospitalier Universitaire de Grenoble, Translational Innovation in Medicine and Complexity, Hôpital Albert Michallon, Centre Hospitalier Universitaire de Clermont-Ferrand, Centre Hospitalier de Béziers, Centre Hospitalier de Fougères, Centre Hospitalier d'Arras, Centre Hospitalier Universitaire de Caen Normandie, Université de Caen Normandie, Centre Hospitalier Intercommunal de Poissy, Hôpital Foch, Centre Hospitalier Universitaire Amiens-Picardie, Centre Hospitalier Universitaire de Reims, Inserm, Université de Bourgogne, CHU Dijon Bourgogne, Interactions Hôte-Greffon-Tumeur & Ingénierie Cellulaire et Génique, Centre Hospitalier Universitaire de Limoges, Hôpital Universitaire Dupuytren, Sorbonne Université, Centre de Recherche Saint-Antoine
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,391
OpenAlex ID: W4286566945