Mortality in Behçet's disease

Area of Science:

• Rheumatology
• Vasculitis Research
• Clinical Epidemiology

Background:

• Behçet's disease (BD) is a rare, chronic inflammatory disorder.
• Understanding long-term mortality is crucial for patient management.

Purpose of the Study:

• To determine the long-term mortality rates and causes of death in a large cohort of patients with Behçet's disease.
• To identify factors associated with increased mortality in BD patients.

Main Methods:

• Analysis of 817 patients diagnosed with BD according to international criteria.
• Assessment of mortality causes, standardized mortality ratios (SMR), and multivariate risk factors.
• Median follow-up of 7.7 years.

Main Results:

• Overall mortality was 5% (41 deaths) over the follow-up period.
• Major causes of death included major vessel disease (43.9%), cancer (14.6%), CNS involvement (12.2%), and sepsis (12.2%).
• Increased mortality was observed in younger adults (15-34 years); male sex, arterial involvement, and frequent disease flares were independent risk factors for mortality.

Conclusions:

• Behçet's disease carries a significant long-term mortality risk, particularly due to vascular complications.
• Male sex, arterial disease, and disease activity (flares) are key predictors of mortality in BD patients.