Abstract
BackgroundPrimary biliary cholangitis (PBC) may progress to cirrhosis and clinically significant portal hypertension (CSPH). This study assesses different features of CSPH and their distinct prognostic impact regarding decompensation and survival in patients with PBC.MethodsPatients with PBC were identified during a database query of our digital patient reporting system.ResultsA total of 333 PBC patients (mean age 54.3 years, 86.8% females, median follow-up 5.8 years) were retrospectively assessed and 127 (38.1%) showed features of CSPH: 63 (18.9%) developed varices, 98 (29.4%) splenomegaly, 62 (18.6%) ascites and 20 (15.7%) experienced acute variceal bleeding. Splenomegaly, portosystemic collaterals and esophageal varices were associated with an increased 5-year (5Y) risk of decompensation (15.0%, 17.8% and 20.9%, respectively). Patients without advanced chronic liver disease (ACLD) had a similar 5Y-transplant free survival (TFS) (96.6%) compared to patients with compensated ACLD (cACLD) but without CSPH (96.9%). On the contrary, PBC patients with cACLD and CSPH (57.4%) or decompensated ACLD (dACLD) (36.4%) had significantly decreased 5Y survival rates. The combination of LSM < 15 kPa and platelets ≥ 150G/L indicated a negligible risk for decompensation (5Y 0.0%) and for mortality (5Y 0.0%). Overall, 44 (13.2%) patients died, with 18 (40.9%) deaths attributed to CSPH-related complications.ConclusionIn PBC, features of CSPH may occur early and indicate an increased risk for subsequent decompensation and mortality. Hence, regular screening and on-time treatment for CSPH is crucial. Combining LSM and platelets serves as a valuable preliminary assessment, as LSM < 15 kPa and platelets ≥ 150G/L indicate an excellent long-term outcome.
Highlights
Primary biliary cholangitis (PBC) is a rare cholestatic liver disease that may progress to cirrhosis [1, 2]
The combination of liver stiffness measurements (LSM) \ 15 kPa and platelets C 150G/L indicated a negligible risk for decompensation (5Y 0.0%) and for mortality (5Y 0.0%)
Since clinically significant portal hypertension (CSPH) drives severe complications [6], such as variceal bleeding and development of ascites, it is of utmost clinical importance to screen for CSPH, as it impacts on prognosis and causes an increased mortality in patients with cirrhosis [7]
Summary
Primary biliary cholangitis (PBC) is a rare cholestatic liver disease that may progress to cirrhosis [1, 2]. Previous studies reported that only a small number of patients showed clinically significant portal hypertension (CSPH) at the time of PBC diagnosis [3,4,5]. Harms et al found that 278 patients with PBC developed CSPH out of a cohort of 3224. According to their results ascites was the most prevalent feature of CSPH, accounting for 63% (N = 175) of all patients diagnosed with CSPH. Primary biliary cholangitis (PBC) may progress to cirrhosis and clinically significant portal hypertension (CSPH).
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