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Primary Biliary Cholangitis

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Abstract

Purpose of review

Primary biliary cholangitis is a progressive autoimmune cholestatic liver disease more commonly affecting middle-aged women. Here we provide an overview of the diagnosis, clinical presentation, management, and prognosis of patients with PBC.

Recent findings

Ursodeoxycholic acid (UDCA) remains the first-line treatment for patients with PBC. Incomplete responders are at risk for disease progression. Obeticholic acid (OCA) is a second-line therapy that has shown benefit in patients with PBC who do not respond to or are intolerant of UDCA, and the off-label use of fibrates continues to show promise, including when used in combination with UDCA and OCA. Newer therapies are under investigation.

Summary

Early diagnosis of PBC combined with the initiation of UDCA remains essential to prevent end-stage liver disease. Risk-stratifying after 1 year of treatment and the need for adjuvant therapy is important to prolong transplant-free survival time.

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Authors and Affiliations

  1. Division of Digestive Health and Liver Diseases, University of Miami Miller School of Medicine, Miami, FL, USA

    Andrew R. Scheinberg MD & Cynthia Levy MD, AGAF, FAASLD

  2. Department of Gastroenterology & Hepatology, Jackson Memorial Hospital / University of Miami Miller School of Medicine, Miami, FL, USA

    Andrew R. Scheinberg MD

  3. Schiff Center for Liver Diseases, 1500 NW 12th Avenue, suite 1101-E, Miami, FL, 33136, USA

    Cynthia Levy MD, AGAF, FAASLD

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  1. Andrew R. Scheinberg MD
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  2. Cynthia Levy MD, AGAF, FAASLD
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Correspondence to Cynthia Levy MD, AGAF, FAASLD.

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Conflict of Interest

Cynthia Levy received research grants from Calliditas, Cara, Cymabay, Escient, Genfit, Gilead, GSK, Intercept, Mitsubishi, Mirum, Novartis, Pliant, Target RWE, and Zydus. She serves as a consultant for Cara, Cymabay, Genfit, Gilead, GSK, Intercept, Mirum, Pliant, Target RWE, and Teva. Andrew R. Scheinberg declares no conflict of interest.

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Scheinberg, A.R., Levy, C. Primary Biliary Cholangitis. Curr Treat Options Gastro 20, 469–483 (2022). https://doi.org/10.1007/s11938-022-00384-z

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