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白塞病的死亡率

Mortality in Behçet's disease.

作者信息

Saadoun D, Wechsler B, Desseaux K, Le Thi Huong D, Amoura Z, Resche-Rigon M, Cacoub P

机构信息

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

出版信息

Arthritis Rheum. 2010 Sep;62(9):2806-12. doi: 10.1002/art.27568.

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.

摘要

目的

报告白塞病(BD)患者的长期死亡率。

方法

对法国一个中心符合BD国际标准的817例患者队列进行死亡原因、标准化死亡率(SMR)及与死亡率相关因素的分析。

结果

在817例BD患者中,41例(5%)在中位随访7.7年后死亡,其中95.1%为男性。死亡时的平均年龄±标准差为34.8±11.9岁。主要死亡原因包括大血管疾病(主要为动脉瘤和布加综合征)(43.9%)、癌症和恶性血液病(14.6%)、中枢神经系统受累(12.2%)及败血症(12.2%)。1年和5年的死亡率分别为1.2%和3.3%。与年龄和性别匹配的健康对照相比,15 - 24岁患者(SMR 2.99,95%置信区间[95%CI] 1.54 - 5.39)和25 - 34岁患者(SMR 2.90,95%CI 1.80 - 4.49)的死亡率增加。35岁以上患者的死亡率降低(SMR 1.23,95%CI 0.75 - 1.92)。多因素分析中,男性(风险比[HR] 4.94,95%CI 1.53 - 16.43)、动脉受累(HR 2.51,95%CI 1.07 - 5.90)及大量BD病情发作(HR 2.37,95%CI 1.09 - 5.14)与死亡风险独立相关。

结论

我们的BD队列中位随访7.7年后的总死亡率为5%。男性、动脉受累及病情发作次数与BD患者的死亡率相关。

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