Evaluation of GLOBE and UK-PBC Scores and Long- Term Outcomes in Primary Biliary Cholangitis Complicated with CREST Syndrome

Abstract Aim Primary biliary cholangitis (PBC) is frequently associated with autoimmune disease. Although there have been some reports of PBC complicated with CREST syndrome (PBC-CREST), the long-term prognosis of these patients has not been fully investigated. Herein, the long-term prognosis of PBC-CREST was compared with that of PBC alone using GLOBE and UK-PBC scores. Methods A total of 302 patients diagnosed with PBC between December 1990 and August 2021 at our hospital and related institutions were included. The survival rates without liver transplantation (LT) were compared between patients with PBC-CREST (n = 57) and those with PBC alone (n = 245). Moreover, 173 patients were divided into two groups (PBC-CREST (n = 26) and PBC alone (n = 147)), excluding those with LT/liver-related death within 1 year after ursodeoxycholic acid administration; GLOBE and UK-PBC scores were compared. Results The survival rates without LT (3/5/10 years) were 98%/96%/96% for the PBC-CREST group and 92%/87%/80% for the PBC-alone group, with a significantly better prognosis in the PBC-CREST group (log-rank, P = 0.0172). The predicted liver-related death and LT risk (5/10/15 years) based on the UK-PBC score was 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 LT-free survival (3/5 years) based on the GLOBE score was significantly higher in the PBC-CREST group (93%/88%) than in the PBC-alone group (88%/81%; P < 0.05). Multivariate analysis revealed that the presence of CREST syndrome is an independent protective factor for the presence of cirrhosis. Conclusions PBC-CREST may have a better long-term prognosis than PBC alone.