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

Semin Arthritis RheumPublished Registry-based cohort study

Main finding · Overall cancerResult — go to the resultRelative risk 1.93 95 % CI 1.03–3.60

Authors' 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

Composed by AI from the published abstract and checked against it. How Briefs are made →

Get them every week

New studies on this topic in My Week and in your Monday e-mail.

We e-mail you a sign-in link and a code. No password, no spam.