Mortality in Behçet's disease.
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.


CONCLUSIONS
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.
Saadoun
D
D
Hôpital Pitié-Salpétrière, Assistance Publique-Hôpitaux de Paris, and Université Pierre et Marie Curie-Paris 6, Paris, France. david.saadoun@psl.aphp.fr
Wechsler
B
B
Desseaux
K
K
Le Thi Huong
D
D
Amoura
Z
Z
Resche-Rigon
M
M
Cacoub
P
P
D000293:Adolescent; D000328:Adult; D000783:Aneurysm / Q000401:mortality / Q000473:pathology; D001013:Aorta, Thoracic / Q000473:pathology; D001528:Behcet Syndrome / Q000150:complications / Q000401:mortality* / Q000473:pathology; D006502:Budd-Chiari Syndrome / Q000401:mortality / Q000473:pathology; D002423:Cause of Death; D015331:Cohort Studies; D015897:Comorbidity; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D008297:Male; D015996:Survival Rate; D055815:Young Adult