Abstract:

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.

Key words: primary Sjogren syndrome, EULAR Sjogren syndrome disease activity index, focus index, interleukin-2 receptor, interleukin-8, tumor necrosis factor α