中文摘要：

目的了解以腺体外器官受累症状首发的原发性干燥综合征（pSS）的发病形式和临床特点。方法收集我院2003年至2006年住院的85例以腺体外表现为首发症状的pSS病例，并对其首发症状及合并症等进行临床分析，对照组为同期住院的87例以口干和（或）眼干为首发症状的pSS病例。结果在85例以腺体外表现为首发症状的pSS患者中，首发症状为关节炎、外周血白细胞减少、血小板减少及肝功能异常的比率分别为44．7％（38／85），18．8％（16／85），10．6％（9／85）和15．3％（13／85），关节炎比率明显高于其他症状（P〈0．05）。与对照组87例以口干和（或）眼干为首发症状的pSS患者相比，以腺体外表现为首发症状的pSS患者的平均确诊时间明显较长[从出现症状至最后确诊平均94．5个月]；血清中自身抗体的阳性率较高。结论pSS发病形式多样，部分患者以腺体外表现为首发症状，其中关节炎所占的比例明显高于其他症状。这些患者部分在病程中出现了口干和（或）眼干的症状，还有少数患者至确诊时仍无口干和（或）眼干的主观症状，这些现象在临床工作中应给予高度重视，以避免误诊。

英文摘要：

Objective To investigate clinical characteristic of primary Sjogren＇s syndrome （pSS） with initial manifestations in the extra-gland organs. Methods Clinical data, including symptoms, laboratory parameters and complications of 85 pSS patients with initial manifestations of extra-gland organs hospitalized at the People＇ s Hospital of Peking University during 2003 to 2006 were collected and analyzed retrospectively, as compared to those of pSS with xerophthalmia or xerostomia as initial ones. Results Mean time interval from onset to diagnosis in these patients with initial manifestations involved in the extra-gland organs was 94. 5 months, higher than that in those with xerophthalmia or xerostomia as initial ones. Arthritis was manifested initially in 38 of the 85 patients （44. 7 percent）, peripheral leukopenia in 16 （ 18. 8 percent）, thrombocytopenia in nine （ 10. 6 percent） and abnormal liver function in 13 （ 15. 3 percent）, respectively. Percentage of serum positive autoantibodies was higher in the patients with initial manifestation of the extra-gland organs than that in those with xerophthalmia or xerostomia as initial ones. Conclusions Initial manifestation of primary Sjogren＇s syndrome varies, and some patients can be initially involved in their extra-gland organs, with more proportion of arthritis than of other symptoms, and xerophthalmia or xerostomia could appear in them during the course of disease. And, xerophthalmia or xerostomia would not appear even as pSS was diagnosed in some other pSS patients. More attention should be paid to these clinical characteristics mentioned above in practice to avoid misdiagnosis.