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Trends in Primary Biliary Cholangitis: Prospective Cohort Study From the European Reference Network Registry
A Gerussi1,2, E Nofit1,2, D P Bernasconi2
1Centre for Autoimmune Liver Diseases and Division of Gastroenterology, European Reference Network (ERN) RARE-LIVER, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.
United European Gastroenterology Journal
|October 22, 2025
Summary
Primary biliary cholangitis (PBC) patients are diagnosed early and treated with Ursodeoxycholic Acid (UDCA). While 85.1% show biochemical response, suboptimal use of second-line therapies for inadequate responders needs improvement.
Area of Science:
- Hepatology
- Gastroenterology
- Rare Diseases
Background:
- Primary biliary cholangitis (PBC) is a rare autoimmune liver disease.
- The European Reference Network on Hepatological Diseases (ERN RARE-LIVER) facilitates research in rare liver conditions.
- Understanding current diagnostic and therapeutic trends in PBC is crucial for optimizing patient care.
Purpose of the Study:
- To evaluate contemporary diagnostic and therapeutic strategies for primary biliary cholangitis (PBC).
- To assess treatment response rates and the utilization of second-line therapies in PBC management.
Main Methods:
- Prospective data from 327 incident PBC cases (2017-2024) were collected from the R-LIVER registry.
- Diagnostic parameters including alkaline phosphatase (ALP) and transient elastography (TE) were analyzed.
- Treatment outcomes with Ursodeoxycholic Acid (UDCA) and second-line therapies were evaluated based on Toronto criteria and ALP normalization at 12 months.
Main Results:
- The majority of PBC patients (89.3%) were female, with a median age of 56 years.
- At diagnosis, median ALP was 1.37 x ULN and median liver stiffness by TE was 6.2 kPa.
- Ursodeoxycholic Acid (UDCA) was initiated in 98.7% of patients, achieving a biochemical response (ALP < 1.67 x ULN) in 85.1% at 12 months. Normalization of ALP occurred in 49.5%.
Conclusions:
- Current PBC diagnosis occurs at an earlier stage, often utilizing non-invasive methods.
- Nearly all PBC patients receive Ursodeoxycholic Acid (UDCA) as first-line therapy, with a high biochemical response rate.
- The application of second-line therapies for inadequate responders in PBC management is currently suboptimal and warrants further investigation.
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