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原发性干燥综合征死亡原因及相关因素分析 |
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曾小峰 吴敏 李明佳 田新平 唐福林 董怡
【摘 要】 目的 了解原发性干燥综合征(pSS)的死亡原因和高危因素。方法 分析172例pSS患者中9例死亡病例的死亡原因，并通过死亡组与存活组各项资料比较， 进行死亡相关单因素分析。结果 死亡9例患者平均存活时间(9.5± 6.3)年，总病死率为5.23%。与pSS相关的最常见的死亡原因为肺间质纤维化伴感染，其他 还有肝硬化腹水感染、血象降低感染、恶性淋巴瘤、颅内出血、肺动脉高压等。9例中与感 染有关的有5例。死亡组与存活组的单因素比较分析发现pSS的死亡高危因素有：男性发病、 肺部受累、浆膜腔积液、雷诺现象及肺动脉高压等。结论 与国外资 料提示pSS患者死亡主要与恶性淋巴瘤相关不同，我国pSS患者最常见的死亡原因为肺间质纤 维化伴感染。
【关键词】 原发性干燥综合征 死亡原因 危险因素 肺纤维化
The causes of death and associated factors in primary Sjgren′s syndro me
ZENG Xiaofeng,WU Min,LI Mingjia,et al.Peking Union Medical College Hospital,Beijing 100730
【Abstract】 Objective To analyze the possible causes of death and associated factors in patients with primary Sjgren′s syn drome (pSS).Methods Nine dead patients in 172 hospitalized with pSS were evaluated and the death associated factors were analyzed by comparison between death group and survival group.Results The average sur vival duration of 9 patients was (9.5±6.3)years.The case-mortality was 5.23 %.The most common cause of death was lung interstitial fibrosis complicated with infection,which finally developed respiratory failure.The other causes were liv er cirrhosis with ascites complicated with infections,pancytopenia,malignant lym phoma,cerebral hemorrhage,pulmonic hypertension.Five of the 9 died patients were associated with infection.The high risk factors of death were male patients,lun g involvement,serositis,Raynaud′s phenomenon and pulmonic hypertension.Conclusion In China,lung interstitial fibrosis complicated with infecti on is the most common cause of death with pSS,which is different from other coun tries′ malignant lymphoma.
【Key words】 Primary Sjgren′s syndrome Cause of death Risk factors Pulmonary fibrosis
原发性干燥综合征(primary Sjgren′s synd-rome，pSS)是一种慢性炎症性的、可累及多系统的自身免疫性疾病，一般认为预后良好，病情可以保持长期稳定，但国内有资料显示我国pSS患者临床表现较国外多且严重［1］，本文对北京协和医院1987—1997[1] [2] [3] 下一页
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