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  • Review Article
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Fatigue in primary biliary cirrhosis

Abstract

Primary biliary cirrhosis (PBC) is a chronic, autoimmune, cholestatic liver disease with a slowly progressive course. Without treatment, most patients eventually develop fibrosis of the liver and may need liver transplantation in the late stage of disease. Fatigue and pruritus are the most common symptoms of PBC, but the majority of patients are asymptomatic at first presentation. There is no specific treatment for fatigue in PBC, but modafinil has shown some potential beneficial effects, such as increased energy levels and decreased total sleep time. This Review article discusses the natural history and the measurement of fatigue in patients with PBC. The central and the peripheral mechanisms that have been suggested for the pathogenesis of fatigue in PBC are also discussed and treatment options are reviewed.

Key Points

  • Fatigue is a common but poorly understood symptom in patients with primary biliary cirrhosis (PBC)

  • The measurement of fatigue has been helped by the development of instruments such as the fatigue impact scale, the PBC-40 survey, and the fatigue severity scale

  • The pathogenesis of fatigue is poorly understood but may be multifactorial, including central nervous system abnormalities, abnormalities of the autonomic nervous system or abnormal levels of cytokines, adipokines, or progesterone metabolites

  • Subclinical depression and peripheral muscle dysfunction may also be associated with the pathogenesis of fatigue in PBC

  • There is no known treatment for the fatigue of PBC although studies of both animals and humans have suggested potential therapeutic avenues for further exploration

  • Patients with fatigue in PBC should have other conditions, such as hypothyroidism, anemia, fibromyalgia, rheumatoid arthritis, systemic lupus erythematosus and depression, considered in the differential diagnosis

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Acknowledgements

The authors acknowledge grant and research support from the American College of Gastroenterology.

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Abbas, G., Jorgensen, R. & Lindor, K. Fatigue in primary biliary cirrhosis. Nat Rev Gastroenterol Hepatol 7, 313–319 (2010). https://doi.org/10.1038/nrgastro.2010.62

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