RECOMPENSATION IS UNCOMMON FOLLOWING INDEX DECOMPENSATION IN PATIENTS WITH DECOMPENSATED PRIMARY BILIARY CHOLANGITIS

INTRODUCTION: Recompensation is associated with improved outcomes in patients with viral and alcohol-related decompensated cirrhosis. However, it is unclear if the concept of recompensation applies in patients with primary biliary cholangitis (PBC) decompensated cirrhosis. We aimed to investigate the prevalence and clinical outcomes of PBC patients with recompensation.

METHODS: We reviewed all decompensated PBC patients diagnosed at the University of Alberta from 1984 to 2022. Biochemical responses were defined based on the Toronto criteria (ALP<1.67 upper normal limit) following at least one year of treatment (ursodeoxycholic acid [UCDA], fibrates or obeticholic acid [OCA]). Recompensation was defined as per the Baveno-VII consensus, which included (1) Resolution of ascites and HE without treatment, (2) Absence of further decompensating events, and (3) Biochemical response by the Toronto criteria, at 1 year following index decompensation. We excluded patients with liver transplantation (LT) or TIPS within a year of index decompensation before the study period. Patients were followed up to death, LT, or most recent visit.

RESULTS: A total of 73 PBC patients with decompensated cirrhosis were identified during the study period. The mean age was 53 years (±13) with female predominance (84%). About 16.4% were decompensated at diagnosis of PBC. The most common index decompensating event was ascites (75%), followed by variceal bleeding (16%) and HE (8.2%). Baseline median (IQR) MELD and Child-Pugh score at diagnosis were 9 (7-11) and 7 (5-8), respectively.
Over a median follow-up of 94 (44-148) months, 47% developed further decompensation. Within a year of index decompensation, 40% died and 78% still required treatment for ascites or HE. Among patients with sufficient data to assess for recompensation (n=53), recompensation occurred in only 5.7% [3/53] patients and exclusively in patients with acute variceal bleeding (100% vs 0%, p<0.001). At 1 year following index decompensation, none of the recompensated patients required LT or died 1 year. However, further decompensation occurred in 2 out of 3 patients (67%).

CONCLUSION: Recompensation is uncommon in decompensated primary biliary cholangitis patients. Index decompensation, especially those unrelated to variceal bleeding, should prompt early LT assessment in PBC patients.