Background Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by lymphocyte infiltration in exocrine glands. Although most of the patients developed the disease in middle age, there are younger onset patients.
Objectives To examine relationship between age at onset and clinical features including disease activity in patients with pSS.
Methods Clinical data of 75 women with pSS who visited our hospital from Aug. 2010 to Dec. 2014 were collected from medical charts. Of them, ESSPRI1 and ESSDAI2 were assessed in 72 and 71 patients, respectively.
Results Average age, age at onset, and duration of disease were 60.9±12.6, 48.3±13.9, and 12.8±7.3 years, respectively. Number of patients with low (ESSDAI <5), moderate (ESSDAI 5 ∼ 13), and high disease activity (ESSDAI 14) were 45, 20, and 6, receptivity. Corticosteroid was used in 60.8% of patients. Average dose of steroid (equivalent dose of prednisolone) was 3.1±2.1 mg/day, and that of high disease activity group (6.3±4.4mg/day) was significantly higher than that of moderate (1.6±1.4 mg/day) or low disease activity group (1.6±1.6mg/day). Patients with younger onset (under 40) had significantly high score of ESSDAI (7.8±5.9) compared with middle age or elderly onset patients (3.7±4.3), and there was a marked difference in its domain of lymphadenopathy between younger onset and middle age or elderly onset patients. There was no difference of ESSPRI between them (5.1±2.1 vs. 4.3±2.1); however, its item of fatigue score was significantly higher in younger onset group than in middle age or elderly onset group (5.9±2.9 vs. 4.3±2.9). Positive rate of anti-Ro/SSA and anti-La/SSB antibody in younger onset group was 100% and 36.8%, respectively and it did not differ from that in middle or elderly onset group (89.3% and 33.9%, respectively).
Conclusions Younger onset pSS patients had higher disease activity than middle age or elderly onset patients. Severity of fatigue and lymphadenopathy were the clinical characteristics in younger onset pSS.
Seror R, et al. Ann Rheum Dis 2011; 70:968-72. doi: 10.1136/ard.2010.143743
Seror R, et al. Ann Rheum Dis 2010;69:1103-9. doi: 10.1136/ard.2009.110619
Disclosure of Interest None declared
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