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Abstract：
Primary biliary cholangitis (PBC) is frequently associated with autoimmune disease. Although PBC complicated with CREST syndrome (PBC-CREST) has been reported, the long-term outcomes of the affected patients have not been fully investigated. Herein, the long-term outcomes of PBC-CREST were evaluated. Next, the GLOBE and UK-PBC scores were validated and compared between the PBC alone and PBC-CREST groups. A total of 302 patients who were diagnosed with PBC between December 1990 and August 2021 at Fukushima Medical University Hospital were included. The liver transplantation (LT)-free survival rates were compared between patients with PBC alone (n = 245) and those with PBC-CREST (n = 57). Moreover, 173 patients, excluding those with liver-related death/LT within 1 year after ursodeoxycholic acid administration, were divided into two subgroups (PBC alone (n = 147) and PBC-CREST (n = 26)), and the GLOBE and UK-PBC scores were compared between the subgroups. The survival rates without LT (3/5/10 years) were 92/87/80% for the PBC-alone group and 98/96/96% for the PBC-CREST group, with a significantly better prognosis in the PBC-CREST group (log-rank P = 0.0172). Multivariate analysis revealed that the presence of CREST syndrome is an independent protective factor for the presence of cirrhosis. The predicted 5/10/15-year risks of liver-related death or LT based on the UK-PBC score were significantly lower in the PBC-CREST group (2.4/7.6/13.2%) than in the PBC-alone group (4.8/11.8/18.8%) (P < 0.05). The predicted 3/5-year LT-free survival rates based on the GLOBE score were significantly higher in the PBC-CREST group (93/88%) than in the PBC-alone group (88/81%) (P < 0.05). Patients with PBC-CREST may have better long-term outcomes than those with PBC alone.
摘要：
原发性胆汁性胆管炎（PBC）通常与自身免疫性疾病有关。虽然PBC并发CREST综合征（PBC-CREST）已有报道，受影响患者的长期结局尚未得到充分调查.在这里，评估了PBC-CREST的长期结局.接下来,对GLOBE和UK-PBC评分进行验证,并在单独PBC组和PBC-CREST组之间进行比较.纳入了1990年12月至2021年8月在福岛医科大学附属医院诊断为PBC的302例患者。比较了单独使用PBC的患者（n=245）和使用PBC-CREST的患者（n=57）的无肝移植（LT）生存率。此外，173名患者，不包括熊去氧胆酸给药后1年内与肝脏相关的死亡/LT，分为两个亚组（单独的PBC（n=147）和PBC-CREST（n=26）），并比较各亚组之间的GLOBE和UK-PBC评分.无LT(3/5/10年)生存率PBC单独组为92/87/80%,PBC-CREST组为98/96/96%,PBC-CREST组的预后明显更好（log-rankP=0.0172）。多因素分析显示，CREST综合征的存在是肝硬化存在的独立保护因素。PBC-CREST组基于UK-PBC评分预测的5/10/15年肝脏相关死亡或LT风险（2.4/7.6/13.2%）明显低于PBC单独组（4.8/11.8/18.8%）（P<0.05）。基于GLOBE评分的3/5年无LT生存率PBC-CREST组(93/88%)明显高于单独PBC组(88/81%)(P<0.05)。PBC-CREST患者可能比单独PBC患者具有更好的长期预后。
