中文摘要：

目的：了解原发性干燥综合征（primary Sjgren’s syndrome,pSS）患者起病形式和临床特点,为临床提供诊断治疗资料。方法：采用流行病学中现况调查的研究方法,对北京大学人民医院风湿免疫科就诊的224例pSS患者进行现况调查,内容包括患者性别、发病年龄、病程、腺体外表现、合并症及家族史,并进一步分析相互间的关系。结果：本组pSS患者平均年龄为（53.5±11.7）岁,男性11例,女性213例,男女之比为1∶19.4,平均发病年龄为（46.1±10.7）岁,中位病程9.4年（0.2～40.0年）。224例患者中确诊pSS前的临床表现中最常见的腺体外表现从高至低依次为白细胞减低74例（33%,74/224）,关节炎56例（25%,56/224）,雷诺现象37例（16.5%,37/224）,肝损伤35例（15.6%,35/224）,肺间质病变27例（12.1%,27/224）,双下肢特发性血小板减少性紫癜26例（11.6%,26/224）,血色素减低18例（8.0%,18/224）,血小板减低13例（5.8%,13/224）,肾小管酸中毒8例（3.6%,8/224）。分析患者脏器受累的影响因素显示,肺间质病变的独立危险因素为：年龄（OR=1.074,95%CI=1.031～1.118）和病程（OR=1.075,95%CI=1.023～1.128）;肝受累的危险因素主要为病程（OR=1.050,95%CI=1.002～1.100）。患者中有自身免疫病家族史者占8.0%（18/224）,有肿瘤家族史者占11.2%（25/224）。结论：pSS患者绝大多数为女性,发病隐匿,病程迁延,容易延误诊断,诊断时多有腺体外脏器受累,尤其是白细胞减低,肝损伤及肺间质病变等严重的系统受累常见,并且该病可能有遗传倾向,应引起临床上更多的重视。

英文摘要：

Objective：To comprehend clinical features at onset of primary Sjgren＇s syndrome（pSS） in order to provide useful data for its clinical management.Methods： In the study,224 patients diagnosed with pSS in the Department of Rheumatology and Immunology of Peking University People＇s Hospital from Jun.1st,2007 to Aug.1st,2008 were investigated,including gender,age of onset,time and site of first hospitalization and definite diagnosis,etc.Results： In this 224 pSS cohort（213 females and 11 males）,the male/female ratio was 1∶19.4,the mean age of onset was（53.5±11.7） years,and median duration was 9.4 years（ranging from 0.2 to 40.0 years）.The manifestations showed that up to 33% of the patients（74/224） had leukopenia,25%（56/224） polyarthralgia,16.5%（37/224） raynaud phenomenon,15.6%（35/224） hepatic injury,12.1%（27/224） pulmonary interstitial fibrosis,11.6%（26/224） purpuras on lower extremities,8.0%（18/224） hemogram abnormal,5.8%（13/224） thrombopenia,and 3.6%（8/224） renal tubule acidosis.When the risk factor of the systemic involvements,was analyzed,two factors were significantly associated with pulmonary interstitial fibrosis： age（OR=1.074,95% CI=1.031-1.118）,and duration（OR=1.075,95% CI=1.023-1.128）.Liver involvement was associated with duration（OR=1.050,95% CI=1.002-1.100）.In addition,8.0% of the pSS patients（18/224）showed family history of autoimmune diseases and 11.2%（25/224）had family history of tumor.Conclusion： In this cohort of the pSS patients,female is predominant and the incidence of extro-glandular manifestations,such as leukopenia,lung and liver involvements is high,and pSS has inheritance intention.