Dr. Cacoub has received consulting fees, speaking fees, and/or honoraria from AstraZeneca, Bristol-Myers Squibb, Sanofi-Aventis, Gilead Sciences, and Schering-Plough (less than $10,000 each) and from Roche and Servier (more than $10,000 each).
Mortality in Behçet's disease
Article first published online: 23 MAY 2010
Copyright © 2010 by the American College of Rheumatology
Arthritis & Rheumatism
Volume 62, Issue 9, pages 2806–2812, September 2010
How to Cite
Saadoun, D., Wechsler, B., Desseaux, K., Huong, D. L. T., Amoura, Z., Resche-Rigon, M. and Cacoub, P. (2010), Mortality in Behçet's disease. Arthritis & Rheumatism, 62: 2806–2812. doi: 10.1002/art.27568
- Issue published online: 31 AUG 2010
- Article first published online: 23 MAY 2010
- Manuscript Accepted: 11 MAY 2010
- Manuscript Received: 14 NOV 2009
To report the long-term mortality in patients with Behçet's disease (BD).
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.
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.
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.