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Mortality in Behçet's disease
D Saadoun1, B Wechsler, K Desseaux
1Hôpital Pitié-Salpétrière, Assistance Publique-Hôpitaux de Paris, and Université Pierre et Marie Curie-Paris 6, Paris, France. david.saadoun@psl.aphp.fr
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Long-term mortality in Behçet
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.
Objective:
To report the long-term mortality in patients with Behçet's disease (BD).
Methods:
A cohort of 817 patients fulfilling the international criteria for BD from a single center in France were analyzed for causes of death, the standardized mortality ratio (SMR), and the factors associated with mortality.
Results:
Among the 817 patients with BD, 41 (5%) died after a median followup of 7.7 years, of whom 95.1% were male. The mean ± SD age at death was 34.8 ± 11.9 years. Main causes of death included major vessel disease (mainly, arterial aneurysm and Budd-Chiari syndrome) (43.9%), cancer and malignant hemopathy (14.6%), central nervous system involvement (12.2%), and sepsis (12.2%). The mortality rate at 1 year and 5 years was 1.2% and 3.3%, respectively. There was an increased mortality among patients ages 15-24 years (SMR 2.99, 95% confidence interval [95% CI] 1.54-5.39) and those ages 25-34 years (SMR 2.90, 95% CI 1.80-4.49) as compared with age-and sex-matched healthy controls. The mortality decreased in patients older than age 35 years (SMR 1.23, 95% CI 0.75-1.92). In multivariate analyses, male sex (hazard ratio [HR] 4.94, 95% CI 1.53-16.43), arterial involvement (HR 2.51, 95% CI 1.07-5.90), and a high number of BD flares (HR 2.37, 95% CI 1.09-5.14) were independently associated with the risk of mortality.
Conclusion:
The overall mortality in our BD cohort was 5% after a median followup of 7.7 years. Male sex, arterial involvement, and the number of flares were associated with mortality in BD.
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