Advancing care in primary biliary cholangitis: emerging insights and novel therapies

Vögelin, Marius; Kremer, Andreas E.

doi:10.1080/14656566.2025.2516622

Cited by 5Liver Diseases and ImmunityPediatric Hepatobiliary Diseases and TreatmentsDrug Transport and Resistance Mechanisms

Advancing care in primary biliary cholangitis: emerging insights and novel therapies

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Vögelin, Marius, Kremer, Andreas E.·2025·Expert Opinion on Pharmacotherapy

DOI 10.1080/14656566.2025.2516622

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Abstract

INTRODUCTION: Primary biliary cholangitis (PBC) is a chronic, cholestatic liver disease, is associated with fatigue and pruritus and can progress to cirrhosis if left untreated. Ursodeoxycholic acid (UDCA) has been the mainstay of therapy for over 40 years. However, 30-40% of PBC patients do not adequately respond to UDCA or have risk factors for disease progression and require second-line treatment. AREAS COVERED: Recent international cohort analyses have provided new insights that enable early identification of high-risk PBC patients and suggest that stricter treatment goals may lower mortality and reduce the need for liver transplantation. Alongside established second-line agents, several promising substances have progressed to phase 2 and 3 trials. Notably, seladelpar and elafibranor, two selective agonists of peroxisome proliferator-activated receptors, achieved high rates of biochemical response and good tolerability, leading to their recent approval for second-line treatment of PBC. Moreover, dedicated clinical trials addressed fatigue and pruritus, the two main symptoms of PBC. EXPERT OPINION: Personalized treatment approaches for PBC are both feasible and essential to improve biochemical response, extend transplant-free survival and alleviate symptom burden. Well-tolerated novel therapies are poised to reshape the treatment landscape in the near future.

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PPAR-δ activation by seladelpar results in bile acid synthesis suppression through downregulating CYP7A1, mediated by FGF21 signaling.
“PPAR-δ activation by seladelpar results in bile acid synthesis suppression, through downregulating cholesterol 7α-hydroxylase (CYP7A1), which is mediated by fibroblast growth factor 21 (FGF21) signaling [45].”
From Second-Line Therapies in Primary Biliary Cholangitis: A Comparative Review of Obeticholic Acid, Fibrates, Seladelpar, and Elafibranor (2025) · cites this paper as [45]
Seladelpar has anti-inflammatory effects on hepatic macrophages and stellate cells, reducing pro-inflammatory cytokines and fibrogenic activity.
“Additionally, it has anti-inflammatory effects on hepatic macrophages and stellate cells, which in turn reduces pro-inflammatory cytokines and fibrogenic activity [44,45,46].”
From Second-Line Therapies in Primary Biliary Cholangitis: A Comparative Review of Obeticholic Acid, Fibrates, Seladelpar, and Elafibranor (2025) · cites this paper as [45]

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“In cases of decompensated liver cirrhosis, caution is advised with the use of estrogens, as they may accumulate due to reduced hepatic metabolism [134].”
Correction to COBALT: A Confirmatory Trial of Obeticholic Acid in Primary Biliary Cholangitis With Placebo and External Controls
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(2024) · PubMed Central · Cited by 2 · see citing works

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Authors: Vögelin, M., & Kremer, A. E.
Affiliations: University Health Network (CA)
University of Zurich (CH)
Corresponding author: Kremer, Andreas E.
ORCID: Kremer, Andreas E.
Venue: Expert Opinion on Pharmacotherapy
Publisher: Informa UK Limited
Published: 2025-06-16
Volume: 26
Issue: 10
Pages: 1149-1162
ISSN: 1465-6566 (ISSN-L)
1744-7666
DOI: 10.1080/14656566.2025.2516622
OpenAlex: W4411191877
Cited by: 5
References: 135
Citations by year: 2026: 2 · 2025: 3
FWCI: 0.73
Type: journal article
Language: en
Open access: hybrid
doi.org
Licence: cc-by-nc-nd
creativecommons.org/licenses/by-nc-nd/4.0/ (vor)
Locations: Expert Opinion on Pharmacotherapy (publishedVersion, cc-by-nc-nd)
PubMed (publishedVersion)
Zurich Open Repository and Archive (University of Zurich) (submittedVersion, cc-by-nc-nd)
Zurich Open Repository and Archive (University of Zurich)
Topics: Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
Health Sciences › Medicine › Oncology › Drug Transport and Resistance Mechanisms
Keywords: primary biliary cirrhosis, ursodeoxycholic acid, second-line therapy, seladelpar, elafibranor, peroxisome proliferator-activated receptor agonists, fatigue, pruritus, transplant-free survival, disease progression, personalized treatment, new drugs
MeSH terms: Animals, Cholagogues and Choleretics, Fatigue, Humans, Liver Cirrhosis, Biliary, Pruritus, Risk Factors, Ursodeoxycholic Acid, Liver Transplantation, Disease Progression, Precision Medicine

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Advancing care in primary biliary cholangitis: emerging insights and novel therapies

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