自身免疫性肝炎(AIH)常并发免疫性疾病,这极大地影响了AIH的病程和临床结局。我们旨在系统地评估临床特征,自身免疫性肝炎伴免疫性疾病的预后。回顾性分析了北京地坛医院358例AIH患者的临床资料。回顾性比较AIH合并免疫性疾病的临床特点,包括临床特征,预后和结果。AIH患者的免疫性疾病患病率为26.5%。结缔组织病(CTD)是与AIH相关的最常见的免疫性疾病(33/358,9.2%),原发性胆汁性胆管炎(PBC)和甲状腺功能异常(TD)的发生率较低(4.7%和8.5%,分别)。诊断时,AIH-PBC患者有较高的IgM和ALP,较低的重量,Hgb,ALT和AFP(P<0.05)。同时,AIH-CTD患者的平均血小板体积较低,血清K和甘油三酯(P<0.05)。AIH-TD患者ANA阳性比例较低(P<0.05)。AIH-TD组的总生存时间明显短于AIH组(P=0.0011)。但AIH-PBC和AIH-CTD没有差异。此外,ANA阴性(HR:0.21,95CI0.13-0.35,P<0.001)可作为预测AIH预后不良的因素,以及AIH-TD患者。大约26.5%的AIH患者患有至少一种免疫疾病,TD与AIH受损患者生存共存。ANA阴性可作为预测AIH和AIH-TD不良预后的独立指标。
Autoimmune hepatitis (AIH) is often complicated with immune diseases, which greatly affected the course and clinical outcome of AIH. We aimed to systematically assess clinical characteristics, prognosis in autoimmune hepatitis accompanied by immune diseases. Clinical records of 358 patients with AIH from Beijing Ditan Hospital in China were retrospectively reviewed. The clinical features of AIH with immune diseases were compared retrospectively, including clinical characteristics, prognosis and outcome. Prevalence of immune diseases in patients with AIH was 26.5%. Connective tissue disease (CTD) was the commonest immune diseases associated with AIH (33/358, 9.2%), and the incidence of primary biliary cholangitis (PBC) and thyroid dysfunction (TD) was low (4.7% and 8.5%, respectively). At diagnosis, AIH-PBC patients had higher IgM and ALP, lower weight, Hgb, ALT and AFP (P < 0.05). Meanwhile, AIH-CTD patients had lower mean platelet volume, serum K and triglyceride (P < 0.05). AIH-TD patients had a lower proportion of ANA positive (P < 0.05). The overall survival time of AIH-TD was significantly shorter than AIH patients (P = 0.0011), but there were no differences in AIH-PBC and AIH-CTD. Furthermore, ANA negative (HR: 0.21, 95%CI 0.13-0.35, P < 0.001) can be a factor to predict the poor prognosis of AIH, and also in AIH-TD patients. About 26.5% of AIH patients had at least one immune disease, and TD coexisted with AIH impaired patients\' survival. ANA negative can be used as an independent indicator to predict the poor prognosis of AIH and AIH-TD.