目的 探讨原发性干燥综合征（pSS）患者的临床特点、疾病活动度及其与细胞因子的相关性。 方法 收集我科住院的pSS患者的临床资料，分别按年龄、性别、唇腺病理分级、疾病活动度分组，回顾性分析其临床特点、细胞因子的差异，及其之间的相关性。 结果 91例pSS患者，男5例，女86例，< 50岁及≥ 50岁患者分别有23、68例，57.14%患者唇腺病理ChisholmⅠ级及以上，腺体外其他器官系统受累约93.41%。腺体外累及中淋巴结肿大最为常见（50.55%），其次为血液系统（45.05%）、球蛋白升高（40.66%）。男性pSS患者主要表现为关节炎/痛、间质性肺炎、血液系统受累、球蛋白升高。< 50岁pSS患者出现发热、补体下降比例高于≥ 50岁pSS患者，但≥ 50岁的pSS患者血液系统受累更常见（P < 0.01），其中粒细胞减少发生率升高。唇腺中重度损害的pSS患者出现球蛋白升高比例高于唇腺轻度损害者。干燥综合征疾病活动指数（ESSDAI）及灶性指数（FS）与年龄呈现负相关。中高疾病活动度组的患者外周血血清白细胞介素?2受体、白细胞介素?8、肿瘤坏死因子?α升高比例明显高于低疾病活动度组。 结论 老年pSS患者更易发生血液系统累及，但总体疾病活动度及唇腺损害会较中青年患者轻微，唇腺中重度损害者更常见唾液腺肿大、球蛋白升高。中高疾病活动度患者外周血血清白细胞介素?2受体、白细胞介素?8、肿瘤坏死因子?α升高更常见。

Objective To investigate the clinical characteristics and disease activity as well as their correlations with cytokine of patients with primary Sjogren syndrome （pSS）. Methods The clinical data of pSS patients hospitalized in our department were retrospectively analyzed. The patients were divided into different groups according to age， gender， pathology of labial gland and disease activity. The differences in clinical characteristics， cytokine and the relations between them were analyzed retrospectively. Results Totally， 91 cases were included in the study. Among them， the ratio between male and female was about 1∶17.2 and 74.73% of the patients were elderly （≥ 50 years） and 25.27% were young and middle-aged （< 50 years）. The labial gland biopsy revealed Chsholm grade I or above in 57.14% of patients. 93.41% of the patients developed extraglandular involvement. Lymphadenopathy was the most common （50.55%） in extraglandular manifestations， followed by pancytopenia （45.05%） and elevated immunoglobulin （40.66%）. The main manifestations of the male pSS patients were arthritis/arthralgia， interstitial pneumonia disease， pancytopenia and elevated immunoglobulin. The young and middle-aged group had higher rates of fever and hypocomplementemia than the elderly group， while the latter had a higher rate of pancytopenia （especially granulocytopenia） than the young and middle-aged group （P < 0.01）. The group with focal lymphocytic sialadenitis had higher rater of globulin elevation than the group without focal lymphocytic sialadenitis. EULAR Sjogren′s syndrome disease activity index （ESSDAI） and FS were negatively correlated with age. The increase rates of serum IL-2R， IL-8， TNFa in the group of moderate and high disease activity were significantly higher than those in the group with low disease activity. Conclusion The elderly pSS patients are more susceptible for pancytopenia， but their disease activity and extraglandular manifestations are less patent and serious， as compared to young and middle-aged patients. The patients with focal lymphocytic sialadenitis are more likely to be affected with salivary gland enlargement and elevated globulin. The increases of serum IL-2R， IL-8 and TNFa are more commonly seen in those patients with moderate and high disease activity.