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Liver Diseases pp 247-259 | Cite as

Primary Biliary Cholangitis

  • Eirini I. RigopoulouEmail author
Chapter

Abstract

Primary Biliary Cholangitis (PBC) is a chronic autoimmune cholestatic liver disease characterised by the presence of antimitochondrial antibodies, which are considered disease-specific and progressive destruction of small intrahepatic bile ducts leading to cholestasis and end-stage liver disease in a proportion of patients.

Since its first description, a considerable amount of data has significantly changed our perception of the disease’s pathogenesis, epidemiology, natural history and treatment.

The geoepidemiology of PBC has changed with prevalence and incidence rates rising over time mainly in developed countries. The strong genetic predisposition of PBC has been revealed by genome-wide association studies, while a large number of environmental factors have emerged as disease triggers over time. Immune mechanisms in PBC are better understood, including the role of biliary epithelial cells in stimulation and maintenance of the inflammatory process.

Treatment aims at delaying disease progression and managing patients’ symptoms. Treatment strategies should be individualized based on patient’s stratification risk as assessed by various prognostic factors. Ursodeoxycholic acid, the mainstay of PBC treatment has in general improved significantly the disease’s natural history, however a proportion of patients don’t respond to treatment. These patients should be offered emerging second-line treatments, including obeticholic acid and other drugs under investigation.

Keywords

Primary biliary cholangitis Antimitochondrial antibodies Treatment Ursodeoxycholic acid Prognostic models 

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Internal Medicine, Faculty of Medicine, School of Health SciencesUniversity of ThessalyLarissaGreece

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