关键词: 原发性胆汁性胆管炎, 临床分型, 自身抗体, 预后

Abstract: Objective The aim of this study was to summarize the features and outcomes of 710 patients with primary biliary cholangitis (PBC) with different clinical classification. Methods A retrospective cohort study was conducted to summarize clinical materials in 710 hospitalized PBC patients admitted to Beijing Ditan hospital between August 2008 and December 2019, and all patients were followed-up for ten years. Results The cohort included 219 patients with chronic progressive type, 224 patients with portal hypertension type and 267 patients with jaundice/liver failure type; patients with jaundice/liver failure type had a significantly higher neutrophil-to-lymphocyte ratio (NLR), total bilirubin (TBIL), gamma-glutamyl transferase (GGT), international normalized ratio (INR), and alkaline phosphatase (ALP) levels compared to those with chronic progressive or portal hypertension types (P<0.05); patients with portal hypertension type had the highest positive rate of anti-centromere antibodies (ACA) (P<0.05); patients with chronic progressive type exhibited the lowest fibrosis 4 score (FIB-4); during the 5-year follow-up, patients with chronic progressive type had significantly lowest incidences of esophageal-gastric varices, ascites and hepatic encephalopathy compared to those with portal hypertension or jaundice/liver failure types (P<0.05); 1-year mortality rates in patients with portal hypertension and jaundice/liver failure types were 1.3% and 5.6%, respectively (P<0.05), while the cumulative 5-year mortality rates were 6.3% and 7.5%, with no significant differences between the two groups (P>0.05); over a 10-year of follow-up, 24 patients with chronic progressive type progressed to portal hypertension type, and 7 progressed to jaundice/liver failure type. Conclusion Among the three clinical types of patients with PBC, the chronic progressive type exhibites the best liver reserve function and prognosis, while those with jaundice/liver failure type is associated with more pronounced cholestasis, severe liver damage, higher short-term mortality and poorer prognosis.

Key words: Primary biliary cholangitis, Clinical classification, Autoantibody, Prognosis