Title :

Serious infections in patients with VEXAS syndrome: data from the French VEXAS registry.

Author(s) :

De Valence, Benjamin [Auteur]
CHU Saint-Antoine [AP-HP]
Delaune, Marion [Auteur]
Institut Universitaire du Cancer de Toulouse - Oncopole [IUCT Oncopole - UMR 1037]
Nguyen, Yann [Auteur]
Hôpital Beaujon [AP-HP]
Jachiet, Vincent [Auteur]
CHU Saint-Antoine [AP-HP]
Heiblig, Mael [Auteur]
Centre Hospitalier Lyon Sud [CHU - HCL] [CHLS]
Jean, Alexis [Auteur]
Centre Hospitalier Universitaire de Bordeaux [CHU Bordeaux]
Riescher Tuczkiewicz, Stanislas [Auteur]
Centre Hospitalier Universitaire de Nantes = Nantes University Hospital [CHU Nantes]
Henneton, Pierrick [Auteur]
Hôpital Saint Eloi [CHU Montpellier]
Guilpain, Philippe [Auteur]
Hôpital Saint Eloi [CHU Montpellier]
Schleinitz, Nicolas [Auteur]
Hôpital de la Timone [CHU - APHM] [TIMONE]
Le Guenno, Guillaume [Auteur]
CHU Estaing [Clermont-Ferrand]
Lobbes, Hervé [Auteur]
CHU Estaing [Clermont-Ferrand]
Lacombe, Valentin [Auteur]
Centre Hospitalier Universitaire d'Angers [CHU Angers]
Ardois, Samuel [Auteur]
Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
Lazaro, Estibaliz [Auteur]
Centre Hospitalier Universitaire de Bordeaux [CHU Bordeaux]
Langlois, Vincent [Auteur]
Outh, Rodreau [Auteur]
Centre Hospitalier Saint Jean de Perpignan
Vinit, Julien [Auteur]
Centre Hospitalier Chalon-sur-Saône William Morey
Martellosio, Jean-Philippe [Auteur]
Centre hospitalier universitaire de Poitiers = Poitiers University Hospital [CHU de Poitiers [La Milétrie]]
Decker, Paul [Auteur]
Centre Hospitalier Universitaire de Nancy [CHU Nancy]
Moulinet, Thomas [Auteur]
Centre Hospitalier Universitaire de Nancy [CHU Nancy]
Dieudonne, Yannick [Auteur]
Centre Hospitalier Universitaire [Strasbourg] [CHU Strasbourg]
Bigot, Adrien [Auteur]
CHU Trousseau [Tours]
Terriou, Louis [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Vlakos, Alexandre [Auteur]
Groupe Hospitalier de La Haute-Saône [GH70]
De Maleprade, Baptiste [Auteur]
CHU Rouen
Denis, Guillaume [Auteur]
Centre Hospitalier de Rochefort [CH Rochefort]
Broner, Jonathan [Auteur]
Centre Hospitalier Universitaire de Nîmes [CHU Nîmes]
Kostine, Marie [Auteur]
Groupe hospitalier Pellegrin
Humbert, Sebastien [Auteur]
Centre Hospitalier Régional Universitaire de Besançon [CHRU Besançon]
Lifermann, François [Auteur]
Centre Hospitalier de Dax
Samson, Maxime [Auteur]
Service d'hématologie biologique [CHU de Dijon]
Pechuzal, Susann [Auteur]
Aouba, Achille [Auteur]
CHU Caen
Kosmider, Olivier [Auteur]
Dion, Jeremie [Auteur]
Institut Universitaire du Cancer de Toulouse - Oncopole [IUCT Oncopole - UMR 1037]
Grosleron, Sylvie [Auteur]
Centre Hospitalier Agen-Nérac
Bourguiba, Rim [Auteur]
CHU Tenon [AP-HP]
Terrier, Benjamin [Auteur]
Hôpital Cochin [AP-HP]
Georgin-Lavialle, Sophie [Auteur]
CHU Tenon [AP-HP]
Fain, Olivier [Auteur]
CHU Saint-Antoine [AP-HP]
Mekinian, Arsène [Auteur]
CHU Saint-Antoine [AP-HP]
Morgand, Marjolaine [Auteur]
CHU Saint-Antoine [AP-HP]
Comont, Thibault [Auteur]
Institut Universitaire du Cancer de Toulouse - Oncopole [IUCT Oncopole - UMR 1037]
Hadjadj, Jérôme [Auteur]
CHU Saint-Antoine [AP-HP]

Journal title :

Annals of the Rheumatic Diseases

Abbreviated title :

Ann Rheum Dis

Publication date :

2023-12-15

ISSN :

1468-2060

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Introduction Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is an acquired autoinflammatory monogenic disease with a poor prognosis whose determinants are not well understood. We aimed to describe ...
Show more >Introduction Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is an acquired autoinflammatory monogenic disease with a poor prognosis whose determinants are not well understood. We aimed to describe serious infectious complications and their potential risk factors. Methods Retrospective multicentre study including patients with VEXAS syndrome from the French VEXAS Registry. Episodes of serious infections were described, and their risk factors were analysed using multivariable Cox proportional hazards models. Results Seventy-four patients with 133 serious infections were included. The most common sites of infection were lung (59%), skin (10%) and urinary tract (9%). Microbiological confirmation was obtained in 76%: 52% bacterial, 30% viral, 15% fungal and 3% mycobacterial. Among the pulmonary infections, the main pathogens were SARS-CoV-2 (28%), Legionella pneumophila (21%) and Pneumocystis jirovecii (19%). Sixteen per cent of severe infections occurred without any immunosuppressive treatment and with a daily glucocorticoid dose ≤10 mg. In multivariate analysis, age >75 years (HR (95% CI) 1.81 (1.02 to 3.24)), p.Met41Val mutation (2.29 (1.10 to 5.10)) and arthralgia (2.14 (1.18 to 3.52)) were associated with the risk of serious infections. JAK inhibitors were most associated with serious infections (3.84 (1.89 to 7.81)) compared with biologics and azacitidine. After a median follow-up of 4.4 (2.5–7.7) years, 27 (36%) patients died, including 15 (56%) due to serious infections. Conclusion VEXAS syndrome is associated with a high incidence of serious infections, especially in older patients carrying the p.Met41Val mutation and treated with JAK inhibitors. The high frequency of atypical infections, especially in patients without treatment, may indicate an intrinsic immunodeficiency.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-02-09T22:16:23Z
2024-03-25T10:33:06Z