CAJ | 학술논문

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.
목적 탐토원발성간조종합정(pSS)환자적림상급면역학특점.방법 회고성분석1985년1월지2005년12월재북경협화의원진치,부합2002년pSS국제분류(진단)표준환자,병채용비삼수검험,t검험화X~2검험여기왕각연구조진행비교.결과 ①573례pSS중녀성점91.4%,평균발병년령현저조우국외환자[(39.0±13.7)세여(52.7±0.9)세,P<0.01].종발병도학진적평균간격시간체48개월.②구간(84.5%)시pSS최상견적증상,기차시안간(70.0%),단균저우국외보도(P<0.01)구간조증、간조성각결막염급진선활검적양성솔고(분별위91.9%、94.8%화90.7%).③pSS합병계통손해자체91.4%,기중발열41.0%、기염4.9%、심포적액14.8%、폐부수루42.3%、신장수루33.5%、갑상선수루32.7%、이선수루5.6%,발생솔균고우국외연구(P<0.01);이핍력、림파결종대급뢰낙현상저우위외연구(P<0.01).④예후위험인소포괄폐동맥고압、고IgM혈증、간공능손해급간질성폐질병.결론 본조pSS환자적발병년령、계통수루상황、자신항체보급사인구성균명현불동우국외환자.폐부화간장손해시중국인pSS예후적위험인소.
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.