#2-66E. Predicting Progression: Identifying Major Organ Involvement Risk in Mucocutaneous Behçet’s Disease (Rheumatology, 2025)

How do we risk-stratify patients presenting with seemingly benign mucocutaneous symptoms of Behçet’s disease (BD) to prevent potentially devastating Major Organ Involvement (MOI)? This international retrospective study utilized the AIDA Network registry data of 328 BD patients who initially presented exclusively with mucocutaneous manifestations, aiming to identify early predictors of progression to MOI (ocular, GI, vascular, or CNS involvement), which occurred in 25% of the cohort over the follow-up period. The core finding reveals that a positive family history for BD and the co-existence of major oral aphthosis combined with minor oral aphthosis, genital aphthosis, or pseudofolliculitis are independently associated with a significantly increased risk of developing MOI. For instance, having both major and minor oral aphthosis was linked to a high odds ratio of 12.76. Critically, clinicians must note that a key limitation is that oral ulcer size classification relied on visual estimation by participating physicians rather than standardized instruments, which introduces a potential for measurement bias. Nevertheless, these data are vital for daily clinical practice, enabling early risk stratification and guiding rigorous long-term monitoring and timely therapeutic intervention for patients predisposed to severe systemic involvement.

Citation: Vitale A, Gavioli F, Caggiano V, et al. Risk of major organ involvement in Behçet’s patients with mucocutaneous onset. Rheumatology. 2025. DOI: 10.1093/rheumatology/keaf613

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