Clinical characteristics and prognosis of Primary biliary cholangitis with esophagogastric varices.

Abstract Background and aims The development of esophagogastric varices (EGV) is one of the most common complications in patients with Primary biliary cholangitis (PBC); yet, factors associated with their development have been poorly characterized. In this cohort, we aimed to investigate the clinical factors associated with EGV in patients with PBC. Methods: The cohort study included patients with PBC who received esophagogastroduodenoscopy screening and ursodeoxycholic acid (UDCA) therapy at baseline. Liver-related death and liver transplantation were assessed as the endpoint, liver transplantation-free survival rates were estimated by using Kaplan–Meier methods, and Cox regression analysis was performed to identify the potential risk factors of endpoint events for PBC with EGV. Results The 690 patients included in this study had a mean age of 57 years old and 553 (80.1%) were females, 13 (7.9%) in the 165 PBC with no liver cirrhosis and 400 (76.2%) in the 525 PBC with liver cirrhosis had signs of EGV, and 215 (31.2%) patients of the cohort had bleeding esophagogastric varices as the first presentation at diagnosis. The median (Interquartile range) follow-up period was 4.0 (2.0, 6.0) years. During the follow-up duration, 99 patients progressed to liver-related deaths, and 23 patients underwent liver transplantation. The 5-year cumulative LT-free survival was 88.20% (95% CI 83.7-92.7) in PBC without EGV, and 80.90% (95% CI 80.0-89.9), 80.30% (95% CI 69.7-90.9), 75.70% (95% CI 68.3-83.1) among PBC with small, medium and large-sized esophageal varices at baseline, respectively (p =0.002). The 10-year cumulative LT-free survival was 68.8% (95% CI 53.9-82.8) in PBC without EGV, and 54.2% (95% CI 36.4-72.0), 51.6% (95% CI 29.9-73.3), 62.1% (95% CI 49.2-75.0) among PBC patients with small, medium and large-sized esophageal varices at baseline, respectively (p=0.001). History of endoscopic therapy and UDCA biochemical responder with Paris criteria for PBC with EGV were associated with a statistically significant reduced risk of LT or death (p =0.001 and p=0.007).Conclusions Endoscopic therapy and a favorable biochemical profile of UDCA can prolong the LT-free survival of PBC patients with EGV, despite the presence of large-sized esophageal varices.