目的:了解青年(≤34岁)原发性干燥综合征(primary Sj gren syndrome,pSS)的发病形式和临床特点。方法:回顾性分析2004~2008年于北京大学人民医院风湿免疫科住院的232例pSS患者的首发症状、临床表现、实验室检查等。结果:青年发病(≤34)患者55例,占总患者数23.7%(55/232),平均年龄(27.6±6.2)岁 中老年发病(〉34岁)患者177例,平均年龄(51.2±14.8)岁。青年组以口干和/或眼干为首发症状的仅占32.73%(18/55),以非口干眼干的腺外器官受累首发的占67.27%(37/55) 中老年组有59.32%(105/177)以口干和/或眼干首发,40.67%(72/177)以腺外器官首发 两组首发症状差异有统计学意义(P=0.001)。青年组从出现症状至确诊为pSS时间明显长于中老年组[(93±107)个月vs(45±59)个月,P=0.001]。青年组患者的系统性损害发生率明显高于中老年组[61.8%(34/55)vs41.24%(73/177),P=0.026]。结论:青年pSS发病患者中,约2/3以非口干和/或眼干为首发症状,出现症状到诊断时间间隔长,系统性损害发生率高于中老年组,在临床工作中应给予充分重视。
Objective:To observe the clinical features of primary Sjogren syndrome (pSS) in young people ( ≤34 years). Methods: The data of 232 patients with pSS who were in hospital from 2004 to 2008 were analysed in a prospective study. Results: A total of 23.7% (55/232)patients' symptoms came on in youth ( ≤34 years), who were all females, mean age was (27. 6 ±6. 2) years. The other 177 patients' symptoms came on when they were more than 34 years old. In young group, 32. 73% (18/ 55) patients' first symptoms were xerostomia and/or keratoconjunctivitis sicca, the other 67. 27% (37/ 55 )came on without xerostomia and keratoconjunctivitis sicca, while in the middle-aged and aged group, 59. 32% (105/177)patients' first symptoms were xerostomia and/or keratoconjunctivitis sicca, 40. 67% ( 72/177 ) without xerostomia and keratoconjunctivitis sicca. There was significant difference ( P = 0. 001 ). The duration from first symptom to first diagnosis of pSS in the young group was (93 ± 107 ) months, which was significantly longer than those of the middle-aged and aged group [ (45 ± 59 ) months, P = 0. 001 ]. The systemic damage of the young group was 61.8% (34/55), which was signifi- cantly greater than 41.24% (73/177) in the middle-aged and aged group( P = 0. 026 ). Conclusion: Pri- mary Sjogren syndrome in young people is not and/or keratoconjunctivitis sicca. Not only the young group is longer, but also the systemic rare, and about 2/3 of them come on without xerostomia duration from first symptom to first diagnosis of pSS in the damage of the young group is greater than those of the mid- dle-aged and aged group. These findings should alert the clinician to make the possible diagnosis of pSS in young patients.