Autoinflammatory & rare systemic diseases
VEXAS syndrome and cancer: Insights about a possible “Tip of the Iceberg”. Ambidirectional data from the international AutoInflammatory Disease Alliance (AIDA) network registries
Main finding · Overall cancerResult — go to the resultRelative risk 1.93 95 % CI 1.03–3.60Authors' conclusion
VEXAS patients exhibit a significantly increased risk of both hematologic and non-hematologic malignancies compared with controls, particularly among those with RP, p.Met41Thr mutation, and persistent systemic inflammation.
Study context
- Population
- Patients aged >60 years from the AIDA Network registries for VEXAS syndrome, Still's disease, BD and Schnitzler's syndrome96 VEXAS and 2181 controls enrolled
- Participants
- 90 vs 174
- Exposure
- VEXAS syndrome vs controls with Still's disease, BD and Schnitzler's syndrome
- VEXASn = 90
- Controlsn = 174reference
- Outcome
- Overall cancer risk (hematologic and non-hematologic neoplasms)
Methodological detailsambidirectional, multicentre international registries (AIDA Network), logistic regression
- Ambidirectional
- Multicentre international registries (AIDA Network)
- Logistic regression
Main finding
Overall cancer
Relative risk
1.93
95 % CI 1.03–3.60
P = 0.036
VEXASvs Controls
RR 1.93 (95 % CI 1.03–3.60); P = 0.036
As reported in the abstract
Observational data: associations, not effects.
Further findings
Factors associated with cancer development (logistic regression, VEXAS patients)
- Relapsing polychondritis: RR (95% CI 1.22-10.64), p = 0.01
- 2.67
- p.Met41Thr mutation: RR (95% CI 1.29-17.33), p = 0.02
- 3.33
- Elevated ESR outside of flares: RR (95% CI 1.01-1.05), p = 0.01
- 1.02
- Lactate dehydrogenase levels outside of flares: RR (95% CI 1.01-1.07), p = 0.04
- 1.02
Original publication
VEXAS syndrome and cancer: Insights about a possible “Tip of the Iceberg”. Ambidirectional data from the international AIDA network registries. Semin Arthritis Rheum. Published 22 January 2026. doi:10. 1016/ j. semarthrit. 2026. 152932
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