Department of Gastroenterology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima City, Fukushima, 960-1295, Japan.
Department of Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Japan.
Sci Rep. 2024 Jun 19;14(1):14124. doi: 10.1038/s41598-024-64976-8.
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.
原发性胆汁性胆管炎（PBC）常与自身免疫性疾病相关。虽然已有报道称 PBC 合并 CREST 综合征（PBC-CREST），但此类患者的长期预后尚未得到充分研究。本研究旨在评估 PBC-CREST 的长期预后，并验证和比较 GLOBE 和 UK-PBC 评分在单纯 PBC 组和 PBC-CREST 组之间的差异。共纳入了 1990 年 12 月至 2021 年 8 月期间在福岛医科大学医院诊断为 PBC 的 302 例患者。比较单纯 PBC 组（n=245）和 PBC-CREST 组（n=57）患者的肝移植（LT）无失败生存率。此外，排除熊去氧胆酸治疗 1 年内发生与肝脏相关的死亡/LT 的 173 例患者，将其分为单纯 PBC 组（n=147）和 PBC-CREST 组（n=26）两个亚组，并比较两组的 GLOBE 和 UK-PBC 评分。单纯 PBC 组和 PBC-CREST 组的 LT 无失败生存率（3/5/10 年）分别为 92/87/80%和 98/96/96%，PBC-CREST 组的预后明显更好（对数秩检验 P=0.0172）。多因素分析显示，存在 CREST 综合征是肝硬化的独立保护因素。基于 UK-PBC 评分预测的 5/10/15 年与肝脏相关的死亡或 LT 风险在 PBC-CREST 组（2.4/7.6/13.2%）显著低于单纯 PBC 组（4.8/11.8/18.8%）（P<0.05）。基于 GLOBE 评分预测的 3/5 年 LT 无失败生存率在 PBC-CREST 组（93/88%）显著高于单纯 PBC 组（88/81%）（P<0.05）。与单纯 PBC 相比，PBC-CREST 患者可能具有更好的长期预后。
