Vasculitis & polymyalgia rheumatica

Risk of major organ involvement in Behçet’s patients with mucocutaneous onset: data from the AutoInflammatory Disease Alliance (AIDA) Network Registry

Rheumatology (Oxford)Published Registry-based cohort study

Authors' conclusion

A positive family history and the presence of major oral aphthosis combined with minor oral aphthosis, genital aphthosis or pseudofolliculitis are associated with MOI development in patients with mucocutaneous BD at onset.

Study context

Population
Patients with exclusively mucocutaneous manifestations at Behçet's disease (BD) onset, AIDA Network registry
Participants
328 patients
Exposure
Clinical features at mucocutaneous BD onset associated with later major organ involvement
Outcome
Development of major organ involvement (MOI)
Follow-up
Entire
Methodological detailsAIDA Network registry
  • AIDA Network registry

Main finding

As reported in the abstract

Development of MOI
Developed major organ involvement (MOI)
82 patients (25%)
Minor oral aphthosis and no major oral aphthosis (reduced risk), OR
0.41 (95% CI 0.22-0.79, P = 0.008)
Both major and minor oral aphthosis, OR
12.76 (95% CI 1.44-113, P = 0.02)
Major oral aphthosis plus genital aphthosis, OR
2.49 (95% CI 1.1-5.6, P = 0.03)
Major oral aphthosis plus pseudofolliculitis, OR
2.9 (95% CI 1.15-7.4, P = 0.02)
Major oral aphthosis plus both genital aphthosis and pseudofolliculitis, OR
3.73 (95% CI 1.22-11.4, P = 0.02)
Positive family history for BD, OR
2.85 (95% CI 1.08-7.58, P = 0.03)

Observational data: associations, not effects.

Original publication

Risk of major organ involvement in Behçet’s patients with mucocutaneous onset: data from the AIDA Network Registry. Rheumatology (Oxford). Published 19 November 2025. doi:10.1093/rheumatology/keaf613

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