Autoinflammatory & rare systemic diseases
Serious infections in patients with VEXAS syndrome: data from the French VEXAS registry
Authors' 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.
Study context
- Population
- Patients with VEXAS syndrome from the French VEXAS Registry133 serious infections
- Participants
- 74 patients
- Outcome
- Serious infectious complications and their potential risk factors
Methodological detailsmulticentre, registry-based, multivariable Cox proportional hazards models
- Multicentre
- Registry-based
- Multivariable Cox proportional hazards models
Main finding
As reported in the abstract
Serious infections: sites, pathogens and risk factors
- Site: lung
- 59%
- Site: skin
- 10%
- Site: urinary tract
- 9%
- Microbiological confirmation
- 76%
- Bacterial
- 52%
- Viral
- 30%
- Fungal
- 15%
- Mycobacterial
- 3%
- Pulmonary infections: SARS-CoV-2
- 28%
- Pulmonary infections: Legionella pneumophila
- 21%
- Pulmonary infections: Pneumocystis jirovecii
- 19%
- Severe infections without any immunosuppressive treatment and with daily GC dose ≤10 mg
- 16%
- Risk factor age >75 years, HR
- 1.81 (1.02 to 3.24) (95% CI)
- Risk factor p.Met41Val mutation, HR
- 2.29 (1.10 to 5.10) (95% CI)
- Risk factor arthralgia, HR
- 2.14 (1.18 to 3.52) (95% CI)
- JAKi (most associated, compared with biologics and azacitidine), HR
- 3.84 (1.89 to 7.81) (95% CI)
Observational data: associations, not effects.
Further findings
Mortality · median follow-up 4.4 (2.5-7.7) years
- Patients died
- 27 (36%)
- Deaths due to serious infections
- 15 (56%)
Original publication
Serious infections in patients with VEXAS syndrome: data from the French VEXAS registry. Ann Rheum Dis. Published 15 February 2024. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →