Primary biliary cirrhosis

• Autores: Margaret Corrigan, Gideon M. Hirschfield
• Localización: Medicine, ISSN-e 1357-3039, Vol. 43, Nº. 11, 2016, págs. 645-647
• Idioma: inglés
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• Resumen

  • Primary biliary cirrhosis (PBC) is an autoimmune liver disease associated with chronic cholestasis and the development of fibrosis and cirrhosis. It is seen most frequently in middle-aged women and a diagnosis is reached in most cases when patients have at least two of the following three features: persistent cholestatic liver biochemistry tests, positive anti-mitochondrial antibody serology and characteristic liver histology.

    Ursodeoxycholic acid (UDCA) is currently the only treatment licensed for use in PBC; most patients respond to treatment and those who fail to respond biochemically have reduced transplant-free survival. Those presenting under the age of 50 years have a 50% chance of treatment failure, and a subsequent high risk of disease progression. Clinical trials are currently evaluating second-line therapies, including novel bile acid therapy, targeted immunosuppression and anti-fibrotic agents. In addition to the potential for progressive liver disease, many patients experience symptoms, particularly pruritus and fatigue, which reduce their quality of life and are associated with significant morbidity. These symptoms are also the focus of attempts to develop novel therapies.