ArticleUnited European gastroenterology journal2025
Trends in Primary Biliary Cholangitis: Prospective Cohort Study From the European Reference Network Registry (R-LIVER).
Article in United European gastroenterology journal, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 4 papers.
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4 citing papers in PubMed.
- Association between body mass index and rheumatoid arthritis: Exploring a possible role for alkaline phosphatase.Medicine · 2026Article
- Trends in Primary Biliary Cholangitis: Prospective Cohort Study From the European Reference Network Registry (R-LIVER).United European gastroenterology journal · 2025Article
- What the European Reference Network Registry for Rare Liver Diseases Tells Us About Primary Biliary Cholangitis in European Practice.United European gastroenterology journal · 2025Article
- Consensus statements of the Hellenic Autoimmune Liver Diseases Study Group on the diagnosis and current management of primary biliary cholangitis.Annals of gastroenterologyArticle
Corrections and comments
- Erratum issued
Authors and funding
24 authors.
Funding
Abstract
BACKGROUND AND
aimsThe European Reference Network on Hepatological Diseases (ERN RARE-LIVER) is a Europe-wide network for centers of excellence in the management of rare liver diseases. We aimed to evaluate the current diagnostic and therapeutic trends of primary biliary cholangitis (PBC).
methodsProspective data of PBC cases diagnosed from 2017 to March 2024 were extracted from the R-LIVER registry of ERN-RARE LIVER. Cases without two follow-ups within 24 months were excluded from the treatment analysis. Biochemical response according to Toronto criteria and normalization of alkaline phosphatase (ALP) values after 12 months of Ursodeoxycholic Acid (UDCA) were evaluated.
resultsThis study included 327 incident cases from six centers. Median age was 56 years, 89.3% were female. At the time of diagnosis, median values of ALP were 1.37 x ULN, and median bilirubin was 0.49 x ULN. Transient elastography (TE) was performed in 230 patients (70.3%) at baseline; median liver stiffness was 6.2 kPa. Out of 316 subjects, treatment with UDCA was started in 312 patients (98.7%); 246 (85.1%) achieved ALP values < 1.67 x ULN at 12 months. Normalization of ALP values occurred in 143 subjects (49.5%) at 12 months. Among 43 patients with inadequate response, 18 (41.9%) were treated with second-line therapies, and had worse liver biochemistry at baseline.
conclusionIn the current era, patients with PBC are diagnosed at an early stage using non-invasive methods and are almost all treated with UDCA. The biochemical response rate is 85.1%, but the use of second-line therapies for inadequate responders remains suboptimal.
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