The Natural History of Deficiency of Adenosine Deaminase 2 Vasculitis in a Large Cohort and Factors Associated With Disease-Related Damage
Study context
- Population
- Patients with DADA2 vasculitis with long-term follow-up
- Participants
- 45 patients
- Outcome
- Factors associated with disease-related damage (Vasculitis Damage Index)
Methodological detailsgenetically homogenous cohort, long-term follow-up, Information collected from patients' charts
- Genetically homogenous cohort
- Long-term follow-up
- Information collected from patients' charts
Main finding
As reported in the abstract
- Young age at disease onset associated with disease-related damage
- Periodic illness with recurrent episodes of fever, myalgia and abdominal pain associated with disease-related damage
Observational data: associations, not effects.
Further findings
Treatment response in severe DADA2 disease
- Patients with severe DADA2 disease responded favorably to tumor necrosis factor inhibitors (TNFi)
Original publication
The Natural History of Deficiency of Adenosine Deaminase 2 Vasculitis in a Large Cohort and Factors Associated With Disease-Related Damage. J Rheumatol. Published 15 January 2026. doi:10.
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