Autoinflammatory & rare systemic diseases
Development of a Disease Activity Index for the Assessment of VEXAS Syndrome (VEXAS‐Disease Activity Index (DAI))
Authors' conclusion
The VEXAS-DAI is a novel instrument designed to quantify active inflammation over time in patients with VEXAS.
Study context
- Population
- Patients with VEXAS syndrome158 test cases
- Outcome
- VEXAS-DAI development
Methodological detailsmodified Delphi methodology, multinational expert advisory committee, consensus defined as ≥75% concurrence, Inflammatory manifestations previously identified through systematic literature review and expert input, Item grading adapted from the Common Terminology Criteria for Adverse Events, Scoring criteria developed on 158 test cases using the PGA of Inflammation
- Modified Delphi methodology
- Multinational expert advisory committee
- Consensus defined as ≥75% concurrence
- Inflammatory manifestations previously identified through systematic literature review and expert input
- Item grading adapted from the Common Terminology Criteria for Adverse Events
- Scoring criteria developed on 158 test cases using the PGA of Inflammation
Recommendations
As reported in the abstract
Consensus achieved after Delphi rounds
4
Domains assessed (inflammatory rash, chondritis, periorbital, ophthalmologic, joint, pulmonary, cardiovascular, genitourinary, neurologic, oral and gastrointestinal, renal, constitutional symptoms)
12
Total score range
0 to 40
Original publication
Development of a Disease Activity Index for the Assessment of VEXAS Syndrome (VEXAS‐DAI). Arthritis Care Res (Hoboken). Published 13 July 2026. doi:10. 1002/ acr. 80117
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