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Cited by 159Liver Diseases and ImmunityPediatric Hepatobiliary Diseases and TreatmentsIgG4-Related and Inflammatory Diseases

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Primary biliary cholangitis: Epidemiology, prognosis, and treatment

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Trivella, Juan, John, Binu V., Levy, Cynthia·2023·Hepatology Communications
DOI 10.1097/hc9.0000000000000179PMC PMC10241503
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Abstract

Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune liver disease characterized by a destructive, small duct, and lymphocytic cholangitis, and marked by the presence of antimitochondrial antibodies. The incidence and prevalence of PBC vary widely in different regions and time periods, and although disproportionally more common among White non-Hispanic females, contemporary data show a higher prevalence in males and racial minorities than previously described. Outcomes largely depend on early recognition of the disease and prompt institution of treatment, which, in turn, are directly influenced by provider bias and socioeconomic factors. Ursodeoxycholic acid remains the initial treatment of choice for PBC, with obeticholic acid and fibrates (off-label therapy) reserved as add-on therapy for the management of inadequate responders or those with ursodeoxycholic acid intolerance. Novel and repurposed drugs are currently at different stages of clinical development not only for the treatment of PBC but also for its symptomatic management. Here, we summarize the most up-to-date data regarding the epidemiology, prognosis, and treatment of PBC, providing clinically useful information for its holistic management.

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Metadata

Authors
Trivella, J., John, B. V., & Levy, C.
Affiliations
  • Medical College of Wisconsin (US)
  • University of Miami (US)
Corresponding author
Levy, Cynthia
ORCID
Venue
Hepatology Communications
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2023-06
Volume
7
Issue
6
ISSN
2471-254X (ISSN-L)
DOI
10.1097/hc9.0000000000000179
PMC
PMC10241503
OpenAlex
W4379184395
Cited by
159
References
42
Citations by year
2026: 43 · 2025: 71 · 2024: 36 · 2023: 9
FWCI
26.88
Type
journal article
Language
en
Open access
Licence
Locations
  • Hepatology Communications (publishedVersion, cc-by-nc-nd)
  • PubMed Central (publishedVersion, cc-by-nc-nd)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
  • Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
  • Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
Keywords
primary biliary cirrhosis, antimitochondrial antibodies, incidence and prevalence, sex differences, racial disparities, prognosis, ursodeoxycholic acid, obeticholic acid, fibrates, drug repurposing, treatment intolerance, socioeconomic factors
MeSH terms
Cholangitis, Cholestasis, Female, Humans, Liver Cirrhosis, Biliary, Male, Prognosis, Ursodeoxycholic Acid

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