摘要
目的 研究原发性胆汁性肝硬化(PBC)、自身免疫性肝炎(AIH)、原发性硬化性胆管炎(PSC)患者中出现其他自身免疫病的比例,并探讨其对预后的影响.方法 收集1994年1月至2014年3月各专科收治的全部自身免疫性肝病(AILD)患者资料共232例,分析对比PBC、AIH和PSC患者合并其他自身免疫病的种类和例数.使用Cox回归模型分析合并其他自身免疫病对AILD预后的影响.结果 232例AILD患者中,PBC患者135例,其中合并干燥综合征(SS) 64例,系统性红斑狼疮(SLE)7例,类风湿关节炎(RA)7例,系统性硬化症(SSc)9例,多发性肌炎(PM)和(或)皮肌炎3例,CD 1例;AIH患者55例,其中合并SS 19例,SLE 10例,RA 1例,SSc 2例,PM和(或)皮肌炎2例;PSC患者24例,其中合并UC 7例,CD 1例,RA 1例;PBC AIH重叠综合征患者18例,其中合并SS 5例,RA1例.调整性别、年龄、病程及药物干预等因素后发现,相比单纯PBC患者,PBC合并SS发生肺间质纤维化的风险明显增加(OR=34.0,95%CI:8.9~130.1).合并其他自身免疫病并未显著影响AILD的预后,包括死亡、肝移植,及肝硬化并发症.结论 AILD合并其他自身免疫病,如SS、SLE、SSc、RA等较为常见.PBC合并SS是发生肺间质纤维化的危险因素,合并其他自身免疫病不独立影响AILD的预后.
Objective To study the probability of other autoimmune diseases in primary biliary cirrhosis (PBC),autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) and explore its effects on the prognosis.Methods From January 1994 to March 2014,the data of 232 patients with autoimmune liver diseases (AILD) were collected.The type and case number of coexisting with other autoimmune diseases of patients with PBC,AIH and PSC were analyzed and compared.Cox regression model was performed to analyze the effects of coexisting with autoimmune diseases on the prognosis of AILD.Results Among 135 PBC patients,there were 64 cases that coexisted with Sjogren's syndrome (SS),seven cases with systemic lupus erythematosus (SLE),seven cases with rheumatoid arthritis (RA),nine cases with systemic sclerosis (SSc),three cases with polymyositis and/or dermatomyositis (PM/DM) and one case with Crohn's disease.Among 55 AIH patients,threre were 19 cases that coexisted with SS,10 cases with SLE,one case with RA,two cases with SSc and two cases with PM/ DM.Among 24 PSC patients,there were seven cases combined with ulceric colitis,one case with Crohn's disease and one case with RA.Among 18 patients with PBC AIH overlap syndrome,there were five cases with SS and one case with RA.Compared with PBC patients,the risk of pulmonary interstitial fibrosis increased in PBC patiento coexisting with SS (OR =34.0,95 % CI 8.9 to 130.1).After gender,age,disease course and medicine intervention were adjusted,the prognosis of AILD which included death,liver transplantation and liver cirrhosis complications was not affected by the coexistence with other autoimmune diseases.Conclusions AILD patients coexisting with other autoimmune diseases is common,most of which are SS,SLE,SSc and RA.PBC patients coexisting with SS is the risk factor of pulmonary interstitial fibrosis,and coexisting with other autoimmune disease does not independently affect the prognosis of AILD.
出处
《中华消化杂志》
CAS
CSCD
北大核心
2015年第2期90-94,共5页
Chinese Journal of Digestion