Assessment of long-term organ damage and predictors of damage accrual in Behçet's syndrome: a 10-year longitudinal study using the Behçet's Syndrome Overall Damage Index.

Abstract

Objectives: To assess long-term organ damage and its accrual in a real-life cohort of Behçet's syndrome (BS) patients using the Behçet's Syndrome Overall Damage Index (BODI), identifying predictors of damage at diagnosis and factors associated with its accrual during follow-up.

Methods: This single-centre retrospective study included 155 BS patients followed for at least six months. Organ damage was assessed at diagnosis and at various timepoints. Damage was defined as a BODI score ≥1 and damage accrual as an increase (ΔBODI) ≥1 between timepoints. Multivariable logistic regressions were used to identify predictors of damage.

Results: At diagnosis, 31.1% of patients already exhibited damage. This prevalence progressively increased to 53.8% at 10-year follow-up, with 39.7% of patients experiencing damage accrual. Pre-existing damage at diagnosis was a risk factor for faster accumulation of new damage. Multivariable analysis identified non-Italian origin and older age at diagnosis as independent predictors of damage at baseline. Notably, older age also remained a significant predictor of damage accrual during the 10-year follow-up. Furthermore, the accumulation of new damage during the disease course was associated with more frequent corticosteroid treatment.

Conclusions: Organ damage in BS is associated with different factors depending on the disease stage: while demographic features, particularly non-Italian origin and older age, significantly predict the presence of damage at diagnosis, older age and corticosteroid therapy are associated with damage accrual in the long-term. These findings highlight the importance of age-targeted clinical monitoring and of steroid-sparing strategies and early intervention in high-risk demographic groups to mitigate irreversible disability.