原发性干燥综合征死亡原因及相关因素分析

The causes of death and associated factors in primary Sjgren′s syndrome

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。

Objective To analyze the possible causes of death and associated factors in patients with primary Sjgren′s syndrome (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 survival 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 liver cirrhosis with ascites complicated with infections,pancytopenia,malignant lymphoma,cerebral hemorrhage,pulmonic hypertension.Five of the 9 died patients were associated with infection.The high risk factors of death were male patients,lung involvement,serositis,Raynaud′s phenomenon and pulmonic hypertension.Conclusion In China,lung interstitial fibrosis complicated with infection is the most common cause of death with pSS,which is different from other countries′ malignant lymphoma.