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Primary Biliary Cholangitis

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Practical Management of Systemic Sclerosis in Clinical Practice

Part of the book series: In Clinical Practice ((ICP))

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Abstract

Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease occurring predominantly in women, which may progress to cirrhosis and its complications. Symptoms are common and may be challenging to treat, and several extra-hepatic conditions are associated. PBC-specific autoantibodies are found in about 25% of patients with systemic sclerosis (SSc), and overt disease has been described in about 10% of cases. As a consequence, PBC should be actively sought in patients with SSc, as early diagnosis and prompt treatment may have a significant impact on disease progression and patient outcomes.

Ursodeoxycholic acid is used routinely in PBC and a biochemical response (observed in 70% of patients) is associated with favourable clinical outcomes. Second-line treatments are now available for those patients who do not respond adequately or are intolerant to ursodeoxycholic acid. Hence, risk-stratification is necessary for optimal clinical management and tailored follow-up. Where multiple co-morbidities exist, a multidisciplinary approach is crucial to manage symptomatology and ensure optimal treatment of individuals.

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Authors and Affiliations

  1. UCL Institute for Liver and Digestive Health and Sheila Sherlock Liver Centre, Royal Free Hospital and UCL, London, UK

    Francesca Saffioti, Douglas Thorburn & Massimo Pinzani

Authors
  1. Francesca Saffioti
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  2. Douglas Thorburn
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  3. Massimo Pinzani
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Correspondence to Massimo Pinzani .

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Editors and Affiliations

  1. University of Florence, Firenze, Firenze, Italy

    Marco Matucci-Cerinic

  2. University College London, Royal Free Hospital, London, UK

    Christopher P. Denton

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Saffioti, F., Thorburn, D., Pinzani, M. (2021). Primary Biliary Cholangitis. In: Matucci-Cerinic, M., Denton, C.P. (eds) Practical Management of Systemic Sclerosis in Clinical Practice. In Clinical Practice. Springer, Cham. https://doi.org/10.1007/978-3-030-53736-4_27

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  • DOI: https://doi.org/10.1007/978-3-030-53736-4_27

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  • Online ISBN: 978-3-030-53736-4

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