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

Dec 11, 2023

Experts: Benjamin de Valence,Marion Delaune,Yann Nguyen,Vincent Jachiet,Mael Heiblig,Alexis Jean,Stanislas Riescher Tuczkiewicz,Pierrick Henneton,Philippe Guilpain,Nicolas Schleinitz,Guillaume Le Guenno,Hervé Lobbes,Valentin Lacombe,Samuel Ardois,Estibaliz Lazaro,Vincent Langlois,Roderau Outh,Julien Vinit,Jean-Philippe Martellosio,Paul Decker,Thomas Moulinet,Yannick Dieudonné,Adrien Bigot,Louis Terriou,Alexandre Vlakos,Baptiste de Maleprade,Guillaume Denis,Jonathan Broner,Marie Kostine,Sebastien Humbert,Francois Lifermann,Maxime Samson,Susann Pechuzal,Achille Aouba,Olivier Kosmider,Jeremie Dion,Sylvie Grosleron,Rim Bourguiba,Benjamin Terrier,Sophie Georgin-Lavialle,Olivier Fain,Arsène Mekinian,Marjolaine Morgand,Thibault Comont,Jerome Hadjadj,

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.
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.
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 2 (28%), (21%) and (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)), 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.
VEXAS syndrome is associated with a high incidence of serious infections, especially in older patients carrying the mutation and treated with JAK inhibitors. The high frequency of atypical infections, especially in patients without treatment, may indicate an intrinsic immunodeficiency.

© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.

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ABOUT THE EXPERTS

Benjamin de Valence,Marion Delaune,Yann Nguyen,Vincent Jachiet,Mael Heiblig,Alexis Jean,Stanislas Riescher Tuczkiewicz,Pierrick Henneton,Philippe Guilpain,Nicolas Schleinitz,Guillaume Le Guenno,Hervé Lobbes,Valentin Lacombe,Samuel Ardois,Estibaliz Lazaro,Vincent Langlois,Roderau Outh,Julien Vinit,Jean-Philippe Martellosio,Paul Decker,Thomas Moulinet,Yannick Dieudonné,Adrien Bigot,Louis Terriou,Alexandre Vlakos,Baptiste de Maleprade,Guillaume Denis,Jonathan Broner,Marie Kostine,Sebastien Humbert,Francois Lifermann,Maxime Samson,Susann Pechuzal,Achille Aouba,Olivier Kosmider,Jeremie Dion,Sylvie Grosleron,Rim Bourguiba,Benjamin Terrier,Sophie Georgin-Lavialle,Olivier Fain,Arsène Mekinian,Marjolaine Morgand,Thibault Comont,Jerome Hadjadj,

Benjamin de Valence

Médecine Interne, Sorbonne université, Hopital Saint-Antoine, Paris, France.

Marion Delaune

Médecine interne, Université Toulouse III-Paul Sabatier Faculté de santé, Centre Hospitalier Universitaire de Toulouse Pole IUC de Toulouse Oncopole CHU, Toulouse, France.

Yann Nguyen

Médecine interne, Université Paris Cité, Hôpital Beaujon, Clichy, France.

Vincent Jachiet

Médecine Interne, Sorbonne université, Hopital Saint-Antoine, Paris, France.

Mael Heiblig

Hématologie clinique, Université Claude Bernard Lyon 1, Centre Hospitalier Lyon-Sud, Pierre-Benite, France.

Alexis Jean

Médecine interne, CHU de Bordeaux, Bordeaux, France.

Stanislas Riescher Tuczkiewicz

Médecine interne, CHU Nantes, Nantes, France.

Pierrick Henneton

Service de Médecine Interne A, Hôpital Saint Eloi, CHRU de Montpellier, Montpellier, France.

Philippe Guilpain

Service de Médecine Interne A, Hôpital Saint Eloi, CHRU de Montpellier, Montpellier, France.

Nicolas Schleinitz

Médecine interne, Aix-Marseille Universite, Hôpital de la Timone, Marseille, France.

Guillaume Le Guenno

Médecine interne, CHU Estaing, Clermont-Ferrand, France.

Hervé Lobbes

Médecine interne, CHU Estaing, Clermont-Ferrand, France.

Valentin Lacombe

Médecine interne et immunologique clinique, CHU Angers, Angers, France.

Samuel Ardois

Médecine interne, CHU Rennes, Rennes, France.

Estibaliz Lazaro

Médecine interne, CHU de Bordeaux, Bordeaux, France.

Vincent Langlois

Médecine interne et infectieuse, Hospital Group Le Havre, Le Havre, France.

Roderau Outh

Service de médecine interne et générale, CH Perpignan, Perpignan, France.

Julien Vinit

Médecine interne, Hospital Centre Chalon-sur-Saon, Chalon-sur-Saone, France.

Jean-Philippe Martellosio

Médecine interne, Centre Hospitalier Universitaire de Poitiers, Poitiers, France.

Paul Decker

Médecine interne et immunologie clinique, CHU de Nancy, Nancy, France.

Thomas Moulinet

Médecine interne et immunologie clinique, CHU de Nancy, Nancy, France.

Yannick Dieudonné

Immunologie Clinique et Médecine Interne, CHU de Strasbourg, Strasbourg, France.

Adrien Bigot

Médecine interne, CHU de Tours, Tours, France.

Louis Terriou

Médecine interne – hématologie, CHU Lille, Lille, France.

Alexandre Vlakos

Médecine interne, Haute-Saône Hospital Group Vesoul Site, Vesoul, France.

Baptiste de Maleprade

Rhumatologie, CHU de Rouen, Rouen, France.

Guillaume Denis

Médecine interne et hématologie, Centre Hospitalier de Rochefort, Rochefort, France.

Jonathan Broner

Médecine interne, CHU Nimes, Nimes, France.

Marie Kostine

Rhumatologie, Centre Hospitalier Universitaire de Bordeaux Groupe hospitalier Pellegrin, Bordeaux, France.

Sebastien Humbert

Hématologie, Centre Hospitalier Universitaire de Besancon, Besancon, France.

Francois Lifermann

Service de médecine interne, CH Dax, Dax, France.

Maxime Samson

Médecine interne, CHU Dijon, Dijon, France.

Susann Pechuzal

Médecine interne-polyvalente, Hôpitaux Drôme Nord, Romans, France.

Achille Aouba

Médecine interne, CHU Caen, Caen, France.

Olivier Kosmider

Service d’Hématologie Biologique, DMU BioPhyGen, APHP, Paris, France.

Jeremie Dion

Médecine interne, Université Toulouse III-Paul Sabatier Faculté de santé, Centre Hospitalier Universitaire de Toulouse Pole IUC de Toulouse Oncopole CHU, Toulouse, France.

Sylvie Grosleron

Médecine interne, Centre Hospitalier Agen-Nérac, Agen, France.

Rim Bourguiba

Médecine interne, CEREMAIA, Sorbonne Université, Hospital Tenon, Paris, France.

Benjamin Terrier

Médecine interne, Université Paris Cité, Hospital Cochin, Paris, France.

Sophie Georgin-Lavialle

Médecine interne, CEREMAIA, Sorbonne Université, Hospital Tenon, Paris, France.

Olivier Fain

Médecine Interne, Sorbonne université, Hopital Saint-Antoine, Paris, France.

Arsène Mekinian

Médecine Interne, Sorbonne université, Hopital Saint-Antoine, Paris, France.

Marjolaine Morgand

Médecine Interne, Sorbonne université, Hopital Saint-Antoine, Paris, France.

Thibault Comont

Médecine interne, Université Toulouse III-Paul Sabatier Faculté de santé, Centre Hospitalier Universitaire de Toulouse Pole IUC de Toulouse Oncopole CHU, Toulouse, France.

Jerome Hadjadj

Médecine Interne, Sorbonne université, Hopital Saint-Antoine, Paris, France jerome.hadjadj@aphp.fr.