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Multicenter Study
. 2024 Feb 15;83(3):372-381.
doi: 10.1136/ard-2023-224819.

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

Benjamin de Valence #  1 , Marion Delaune #  2 , Yann Nguyen  3 , Vincent Jachiet  1 , Mael Heiblig  4 , Alexis Jean  5 , Stanislas Riescher Tuczkiewicz  6 , Pierrick Henneton  7 , Philippe Guilpain  7 , Nicolas Schleinitz  8 , Guillaume Le Guenno  9 , Hervé Lobbes  9 , Valentin Lacombe  10 , Samuel Ardois  11 , Estibaliz Lazaro  5 , Vincent Langlois  12 , Roderau Outh  13 , Julien Vinit  14 , Jean-Philippe Martellosio  15 , Paul Decker  16 , Thomas Moulinet  16 , Yannick Dieudonné  17 , Adrien Bigot  18 , Louis Terriou  19 , Alexandre Vlakos  20 , Baptiste de Maleprade  21 , Guillaume Denis  22 , Jonathan Broner  23 , Marie Kostine  24 , Sebastien Humbert  25 , Francois Lifermann  26 , Maxime Samson  27 , Susann Pechuzal  28 , Achille Aouba  29 , Olivier Kosmider  30 , Jeremie Dion  2 , Sylvie Grosleron  31 , Rim Bourguiba  32 , Benjamin Terrier  33 , Sophie Georgin-Lavialle  32 , Olivier Fain  1 , Arsène Mekinian  1 , Marjolaine Morgand  1 , Thibault Comont #  2 , Jerome Hadjadj #  34 ; French VEXAS Group
Collaborators, Affiliations
Multicenter Study

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

Benjamin de Valence et al. Ann Rheum Dis. .

Abstract

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.

Keywords: Glucocorticoids; Immune System Diseases; Inflammation; Therapeutics; Tumor Necrosis Factor Inhibitors.

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Conflict of interest statement

Competing interests: None declared.

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Supplementary concepts