Mortality in Behçet's disease

Authors

  • D. Saadoun,

    Corresponding author
    1. Hôpital Pitié-Salpétrière, Assistance Publique-Hôpitaux de Paris, and Université Pierre et Marie Curie-Paris 6, Paris, France
    • Service de Médecine Interne, AP-HP, Hôpital Pitié-Salpêtrière, Service de Médecine Interne, 83 Boulevard de l'Hôpital, Paris F-75013, France
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  • B. Wechsler,

    1. Hôpital Pitié-Salpétrière, Assistance Publique-Hôpitaux de Paris, and Université Pierre et Marie Curie-Paris 6, Paris, France
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  • K. Desseaux,

    1. INSERM U717, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France
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  • D. Le Thi Huong,

    1. Hôpital Pitié-Salpétrière, Assistance Publique-Hôpitaux de Paris, and Université Pierre et Marie Curie-Paris 6, Paris, France
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  • Z. Amoura,

    1. Hôpital Pitié-Salpétrière, Assistance Publique-Hôpitaux de Paris, and Université Pierre et Marie Curie-Paris 6, Paris, France
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  • M. Resche-Rigon,

    1. INSERM U717, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France
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  • P. Cacoub

    1. Hôpital Pitié-Salpétrière, Assistance Publique-Hôpitaux de Paris, and Université Pierre et Marie Curie-Paris 6, Paris, France
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    • 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).


Abstract

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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