Pediatric rheumatology

Von Willebrand factor antigen as a marker of disease activity in childhood-onset antineutrophil cytoplasmic antibody–associated vasculitis

Rheumatology (Oxford)Published Retrospective cohort study

Main finding · VWF antigen levelResult — go to the resultOdds ratio 3.01 95% CI 2.3–3.93

Authors' conclusion

Increased VWF antigen levels correlate with active vasculitis in this paediatric-onset AAV cohort

Study context

Population
Children with ANCA-associated vasculitis (AAV) with more than one Von Willebrand factor (VWF) level measured732 VWF measurements
Participants
33 children
Exposure
Active vs inactive disease
Outcome
Relationship between VWF antigen and disease activity over time
Methodological detailsgeneralized estimating equations, repeated measures correlation, ROC analysis
  • Generalized estimating equations
  • Repeated measures correlation
  • ROC analysis

Main finding

VWF antigen level

Odds ratio

3.01

95% CI 2.3–3.93

P < 0.001

By group

Active diseasevs Inactive disease
OR 3.01 (95% CI 2.3–3.93); P < 0.001

As reported in the abstract

Observational data: associations, not effects.

Further findings

VWF effect by disease subtype

No substantial difference between disease subtypes

Correlation of VWF antigen with disease activity and diagnostic performance

Moderate positive correlation, with acceptable sensitivity and specificity

Original publication

Von Willebrand factor antigen as a marker of disease activity in childhood-onset antineutrophil cytoplasmic antibody–associated vasculitis. Rheumatology (Oxford). Published 14 November 2023. doi:10.1093/rheumatology/kead600

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