Jayabalan Dujinthan, Huang Yi, Calzadilla-Bertot Luis, Adams Leon A, Cheng Wendy, Hazeldine Simon, Smith Briohny W, MacQuillan Gerry C, Wallace Michael C, Garas George, Jeffrey Gary P
Department of Hepatology, Sir Charles Gairdner Hospital, Nedlands, Western Australia, Australia.
Medical School, The University of Western Australia, Nedlands, Western Australia, Australia.
J Gastroenterol Hepatol. 2025 Mar;40(3):731-740. doi: 10.1111/jgh.16850. Epub 2024 Dec 17.
Prognosis in autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) has historically been poor. This multicenter retrospective cohort study investigated the natural history and assessed the predictors of outcomes in patients with AIH, PBC, and PSC.
AIH, PBC, and PSC patients were identified from the state-wide Hepascore and Clinical Outcome cohort. Overall death or transplant, liver-related mortality (liver-related death or transplant), and liver-related decompensation were determined using a population-based data linkage system. Baseline Liver Outcome Score (LOS), Hepascore, and MELD were examined for predicting outcomes.
Two-hundred thirty-seven AIH patients (24% male, median age 56.6 years [range, 14.3-94.0]), 157 PBC patients (8.3% male, median age 60.5 years [range, 25.6-87.1]), and 167 PSC patients (52.7% male, median age 55.6 years [range, 18.4-88.6]) were enrolled. Five-year transplant-free survival was 88% (95%CI: 81-92%) in AIH, 92% (95%CI: 85-96%) in PBC, and 61% (95%CI: 51-69%) in PSC. PSC had a significantly worse overall death or transplant, liver-related mortality, and liver-related decompensation when compared to AIH and PBC (p < 0.0001). LOS was a significant independent predictor of overall death or transplant, liver-related mortality, and liver-related decompensation among patients with AIH and PBC. LOS was a significant independent predictor of overall death or transplant in patients with PSC, and Hepascore was a significant independent predictor of liver-related mortality and liver-related decompensation.
Outcomes for AIH and PBC are excellent but remain poor in PSC. LOS is a predictor of outcomes in autoimmune liver disease.
自身免疫性肝炎（AIH）、原发性胆汁性胆管炎（PBC）和原发性硬化性胆管炎（PSC）的预后历来较差。这项多中心回顾性队列研究调查了AIH、PBC和PSC患者的自然病史，并评估了其预后的预测因素。
从全州范围的肝评分和临床结果队列中识别出AIH、PBC和PSC患者。使用基于人群的数据链接系统确定总体死亡或移植、肝脏相关死亡率（肝脏相关死亡或移植）以及肝脏相关失代偿情况。检查基线肝脏结局评分（LOS）、肝评分和终末期肝病模型（MELD）以预测预后。
纳入了237例AIH患者（男性占24%，中位年龄56.6岁[范围14.3 - 94.0岁]）、157例PBC患者（男性占8.3%，中位年龄60.5岁[范围25.6 - 87.1岁]）和167例PSC患者（男性占52.7%，中位年龄55.6岁[范围18.4 - 88.6岁]）。AIH患者的5年无移植生存率为88%（95%置信区间：81 - 92%），PBC患者为92%（95%置信区间：85 - 96%），PSC患者为61%（95%置信区间：51 - 69%）。与AIH和PBC相比，PSC的总体死亡或移植、肝脏相关死亡率以及肝脏相关失代偿情况明显更差（p < 0.0001）。LOS是AIH和PBC患者总体死亡或移植、肝脏相关死亡率以及肝脏相关失代偿的显著独立预测因素。LOS是PSC患者总体死亡或移植的显著独立预测因素，肝评分是肝脏相关死亡率和肝脏相关失代偿的显著独立预测因素。
AIH和PBC的预后良好，但PSC的预后仍然较差。LOS是自身免疫性肝病预后的一个预测因素。
