【摘要】 目的探讨自身免疫性肝病并发系统性自身免疫性疾病患者的临床特点及预后,为提高该类疾病的诊治水平提供依据。方法对我院2011年3月至2015年4月收治的170例自身免疫性肝病患者的临床特征及生存资料进行回顾性分析,比较原发性胆汁性胆管炎(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性疾病的比例和种类,采用Cox回归模型探讨并发系统性自身免疫性疾病对自身免疫性肝病患者预后的影响。结果自身免疫性肝病患者的好发年龄在41～60岁之间;在不同类型自身免疫性肝病患者中,以乏力、恶心、瘙痒、纳差这4种的临床症状具有统计学差异(P<0. 05);PBC、AIH和重叠综合征(OS)组并发干燥综合征、类风湿关节炎、Ⅰ型糖尿病、甲减/甲亢、系统性红斑狼疮等系统性自身免疫性疾病的比例显著高于PSC组,但并发溃疡性结肠炎的比例显著低于PSC组(P<0. 05)。170例患者随访期间并发症发生率:食管胃底静脉曲张破裂出血(15例,8. 82%)、腹水(8例,4. 71%)、肝性脑病(4例,2. 35%)、肝细胞癌(2例,1. 18%);死亡(17例,6. 47%),其中肝病相关死亡(6例,3. 53%)。并发与不并发系统性自身免疫性疾病的自身免疫性肝病患者相比,其1年和3年生存率无明显统计学差异(P>0. 05)。结论自身免疫性肝病患者并发系统性自身免疫性疾病中以干燥综合征、类风湿关节炎、Ⅰ型糖尿病、甲减/甲亢、系统性红斑狼疮等较为常见。并发系统性自身免疫性疾病的自身免疫性肝病患者预后差,但合并其他自身免疫病并不独立影响自身免疫性肝病患者的预后。更多还原

【Abstract】 Objective To explore the clinical features and the prognosis of autoimmune liver disease complicated by systemic autoimmune diseases.Methods A retrospective analysis was made to the clinical features and survival data of170 cases with autoimmune liver disease hospitalized during the period from Mar.,11,2011 to April,2015; the proportions and types of autoimmune disease incidence were comparatively studied among primary biliary cholangitis(PBC),autoimmune hepatitis(AIH),and primary sclerosing cholangitis(PSC); Cox regression model was applied in investigating the effect the complicated systemic autoimmune disease on the prognosis of the cases with autoimmune live disease.Results The peak incidence age of autoimmune disease was from 41 to 60 years; the 4 clinical symptoms:itching,fatigue,nausea,and anorexia,in cases with different types of autoimmune liver diseases had statistical difference(P<0. 05); the complication proportions of Sjogren syndrome,rheumatoid arthritis,type I diabetes,hypothyroidism,hyperthyroidism,and systemic lupus erythematosus in cases with PBC,AIH and Overlapping syndrome(OS)were higher than those in cases with PSC while that of ulcerative colitis was lower; the incidence of complications during follow-up included 8. 82%(15/170) of esophageal varices bleeding,4. 71%(8/170) of ascites,2. 35%(4/170) of hepatic encephalopathy and 1. 18%(2/170) of hepatocellular carcinoma; 17 cases(6. 4%) died including the 6 cases who died from related liver diseases; the 1-year survival and 3-yrar survival of the cases with autoimmune liver disease complicated by systemic autoimmune diseases were of no obvious statistical difference from those of the cases with autoimmune liver disease complicated by no systemic autoimmune diseases(P > 0. 05). Conclusions It is common for cases with autoimmune liver disease to have the complications of Sjogren syndrome, rheumatoid arthritis,type I diabetes,hypothyroidism/hyperthyroidism and systemic lupus erythematosus; the prognosis of the cases with autoimmune liver disease complicated by systemic autoimmune diseases is poor while the combination with other autoimmune diseases is of no effect on the prognosis of the cases with autoimmune liver disease.更多还原