Autoinflammatory & rare systemic diseases

Comparing clinical features between males and females with VEXAS syndrome: data from literature analysis of patient reports

Rheumatology (Oxford)Published Systematic literature review with pooled analysis of individual patient reports

Authors' conclusion

Our results supported the hypothesis that the UBA1 mutation should be sought under the same conditions in both sexes.

Study context

Population
Patients with VEXAS syndrome with precise, individual VEXAS-related features described in previously published clinical descriptions104 articles
Participants
224 patients
Comparison
Females vs males with VEXAS syndrome
  • Womenn = 9
  • Menn = 215reference
Outcome
Clinical and biological features of VEXAS syndrome by sex
Methodological detailsliterature-based cohort, individual patient data
  • Literature-based cohort
  • Individual patient data

Pooled result

As reported in the abstract

Clinical and biological features by sex
  • No difference was observed in the clinical or biological features according to sex

Further pooled results

Type of UBA1 mutation and association with myelodysplastic syndrome

No difference in the type of UBA1 mutation or the association with myelodysplastic syndrome

Karyotype among women

Constitutional 45,X karyotype
1
Acquired X monosomy in bone marrow karyotype
4
Marrow karyotype not provided

Original publication

Comparing clinical features between males and females with VEXAS syndrome: data from literature analysis of patient reports. Rheumatology (Oxford). Published 24 February 2024. doi:10.1093/rheumatology/keae123

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