原发性干燥综合征573例临床分析

目的 探讨原发性干燥综合征(pSS)患者的临床及免疫学特点.方法 回顾性分析1985年1月至2005年12月在北京协和医院诊治,符合2002年pSS国际分类(诊断)标准患者,并采用非参数检验,t检验和X~2检验与既往各研究组进行比较.结果 ①573例pSS中女性占91.4%,平均发病年龄显著早于国外患者[(39.0±13.7)岁与(52.7±0.9)岁,P<0.01].从发病到确诊的平均间隔时间达48个月.②口干(84.5%)是pSS最常见的症状,其次是眼干(70.0%),但均低于国外报道(P<0.01)口干燥症、干燥性角结膜炎及唇腺活检的阳性率高(分别为91.9%、94.8%和90.7%).③pSS合并系统损害者达91.4%,其中发热41.0%、肌炎4.9%、心包积液14.8%、肺部受累42.3%、肾脏受累33.5%、甲状腺受累32.7%、胰腺受累5.6%,发生率均高于国外研究(P<0.01);而乏力、淋巴结肿大及雷诺现象低于围外研究(P<0.01).④预后危险因素包括肺动脉高压、高IgM血症、肝功能损害及间质性肺疾病.结论 本组pSS患者的发病年龄、系统受累状况、自身抗体谱及死因构成均明显不同于国外患者.肺部和肝脏损害是中国人pSS预后的危险因素.

The clinical characteristics of 573 cases of primary Sj(o)gren's syndrome

Objective To analyze the clinical manifestations, immunological features and prognosis of primary Sj(o)gren's syndrome (pSS). Methods Five hundred and seventy-three patients who fulfilled the 2002 international classification (criteria) for pSS from Peking Union Medical College Hospital between 1985 and 2005 were screened retrospectively and compared with others. T-test, nonparametric test and Chi-square test were used data analysis. Results ① Among the 573 cases of pSS patients, women accounted for 91.4%. Mean age of onset was significantly earlier than foreign patients [(39.0±13.7) yrs vs (52.7+0.9) yrs, P<0.01]. The average duration from disease onset to pSS diagnosis was 48 mo. ② Dry mouth (84.5%) and dry eyes (70.0%) were the most common symptoms, somehow lower than foreign patients (P<0.0l). The positivity of xerophthalmia, xerostomia and salivary gland biopsy were high (91.9%, 94.8% and 90.7%, respectively). ③ 91.4% patients suffered from systemic involvement. Compared with other foreign studies, incidence of fever 41.0%, myositis 4.9%, pericardial effusion 14.8%, pulmonary involvement 42.3%, renal involvement 33.5%, thyroid involvement 32.7% and pancrease involvement 5.6% (P<0.01) was significantly high, while the incidence of fatigue, lymphadenectasis and Raynaud's phenomenon (P<0.01) was significantly lower. ④ Risk factor for poor prognosis was as following: pulmonary artery hypertension, liver damage and interstitial lung disease. Conclusion Chinese pSS isdifferent from those foreign countries in age of onset, systemic involvement, autoantibodies and proportional mortality rate. Lung and liver damage are the high risk factors for poor disease prognosis.