Autoinflammatory & rare systemic diseases

Development of a Disease Activity Index for the Assessment of VEXAS Syndrome (VEXAS‐Disease Activity Index (DAI))

Arthritis Care Res (Hoboken)Published Consensus-based instrument development study

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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