Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver.[1][2][3] It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis. Further slow damage to the liver tissue can lead to scarring, fibrosis, and eventually cirrhosis.
Common symptoms are tiredness, itching, and in more advanced cases, jaundice. In early cases, the only changes may be those seen in blood tests.[4]
PBC is a relatively rare disease, affecting up to one in 3,000–4,000 people.[5][6] As with many other autoimmune diseases, it is much more common in women,[7] with a sex ratio of at least 9:1 female to male.[1] The reasons for this disparity are unclear, but may involve the expression of sex hormones such as estrogen, which impact immune system response.[7]
The condition has been recognised since at least 1851, and was named "primary biliary cirrhosis" in 1949.[8] Because cirrhosis is a feature only of advanced disease, a change of its name to "primary biliary cholangitis" was proposed by patient advocacy groups in 2014.[9][10]
^ abPoupon R (May 2010). "Primary biliary cirrhosis: a 2010 update". Journal of Hepatology. 52 (5): 745–758. doi:10.1016/j.jhep.2009.11.027. PMID 20347176.
^Hirschfield GM, Gershwin ME (January 2013). "The immunobiology and pathophysiology of primary biliary cirrhosis". Annual Review of Pathology. 8: 303–330. doi:10.1146/annurev-pathol-020712-164014. PMID 23347352.
^Dancygier H (2010). Principles and Practice of Clinical Hepatology. Springer. pp. 895–. ISBN 978-3-642-04509-7. Retrieved 29 June 2010.
^Cite error: The named reference Lancet2011 was invoked but never defined (see the help page).
^Cite error: The named reference pmid22245904 was invoked but never defined (see the help page).
^Cite error: The named reference pmid10421645 was invoked but never defined (see the help page).
^ abMoulton, Vaishali R. (2018). "Sex Hormones in Acquired Immunity and Autoimmune Disease". Frontiers in Immunology. 9: 2279. doi:10.3389/fimmu.2018.02279. ISSN 1664-3224. PMC 6180207. PMID 30337927.
^Dauphinee JA, Sinclair JC (July 1949). "Primary biliary cirrhosis". Canadian Medical Association Journal. 61 (1): 1–6. PMC 1591584. PMID 18153470.
^PBC Foundation (UK). "PBC Name Change". Retrieved 27 Jan 2017.
^"Primary Biliary Cirrhosis Name Change Initiative" (PDF). PBCers Organization. Archived from the original (PDF) on April 19, 2015. Retrieved September 15, 2022.
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