Mucocutaneous-onset Behçet’s disease: distinct early patterns, distinct long-term risks

This editorial refers to the article ‘Risk of major organ involvement in Behçet’s patients with mucocutaneous onset: data from the AIDA Network Registry’ by Antonio Vitale et al. 2025; https://doi.org/10.1093/rheumatology/keaf613. Can we predict which patients presenting with exclusively mucocutaneous Behçet’s disease (BD) will later develop major organ involvement (MOI)? This question is frequently encountered in daily clinical practice because the long-term systemic trajectory of patients who present with apparently mucocutaneous-limited disease at onset remains difficult to anticipate. The lack of validated markers creates uncertainty when clinicians must decide how closely to monitor and how intensively to treat patients. As BD may evolve over time with the emergence of MOI in patients initially presenting with mucocutaneous disease alone, identifying early risk signals remains an important need in both adults and children [1, 2]. In a recent study published in Rheumatology, Vitale et al. [3] address this clinically relevant uncertainty by using data from the international AIDA Network registry to quantify the long-term risk of MOI among patients with mucocutaneous-onset BD and to identify baseline mucocutaneous patterns associated with future risk.