Editing Primary biliary cholangitis (section)

{{Short description|Autoimmune disease of the liver}}
{{Use dmy dates|date=August 2024}}
{{cs1 config |name-list-style=vanc |display-authors=6}}
{{Infobox medical condition (new)
| name            = Primary biliary cholangitis
| image           = Primary biliary cirrhosis intermed mag much cropping.jpg
| caption         = [[Micrograph]] of PBC showing [[bile duct]] [[inflammation]] and injury, [[H&E stain]]
| pronounce       =
| field           = [[Gastroenterology]], [[Hepatology]]
| synonyms        = Primary biliary cirrhosis
| symptoms        = [[Cholestasis]], [[pruritus]], [[fatigue (medical)|fatigue]]
| complications   = [[Cirrhosis]], [[hepatic failure]], [[portal hypertension]]
| onset           = Usually middle-aged women
| duration        =
| types           =
| causes          = Autoimmune
| risks           = Female sex
| diagnosis       = [[Anti-mitochondrial antibodies]], <br />[[Liver biopsy]]
| differential    = [[Autoimmune hepatitis]]
| prevention      =
| treatment       = [[Ursodeoxycholic acid]], [[obeticholic acid]], [[cholestyramine]]
| medication      =
| prognosis       =
| frequency       = 1 in 3,000–4,000 people
| deaths          =
}}

'''Primary biliary cholangitis''' ('''PBC'''), previously known as '''primary biliary cirrhosis''', is an [[autoimmune disease]] of the [[liver]].<ref name="pmid20347176">{{cite journal | vauthors = Poupon R | title = Primary biliary cirrhosis: a 2010 update | journal = Journal of Hepatology | volume = 52 | issue = 5 | pages = 745–758 | date = May 2010 | pmid = 20347176 | doi = 10.1016/j.jhep.2009.11.027 | doi-access = free }}</ref><ref name="pmid23347352">{{cite journal | vauthors = Hirschfield GM, Gershwin ME | title = The immunobiology and pathophysiology of primary biliary cirrhosis | journal = Annual Review of Pathology | volume = 8 | pages = 303–330 | date = January 2013 | pmid = 23347352 | doi = 10.1146/annurev-pathol-020712-164014 }}</ref><ref name="Dancygier2010">{{cite book | vauthors = Dancygier H |title= Principles and Practice of Clinical Hepatology|url=https://books.google.com/books?id=lrPX8C4p90QC&pg=PA895 |access-date=29 June 2010 |year=2010 |publisher=Springer |isbn=978-3-642-04509-7 |pages=895–}}</ref> It results from a slow, progressive destruction of the small [[bile duct]]s 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]].

<!-- Symptoms -->
Common symptoms are [[Fatigue (physical)|tiredness]], [[pruritus|itching]], and in more advanced cases, [[jaundice]]. In early cases, the only changes may be those seen in blood tests.<ref name=Lancet2011/>

<!-- Epidemiology -->
PBC is a relatively rare disease, affecting up to one in 3,000–4,000 people.<ref name="pmid22245904" /><ref name="pmid10421645" />  As with many other autoimmune diseases, it is much more common in women,<ref name=":1">{{Cite journal |last=Moulton |first=Vaishali R. |date=2018 |title=Sex Hormones in Acquired Immunity and Autoimmune Disease |journal=Frontiers in Immunology |volume=9 |pages=2279 |doi=10.3389/fimmu.2018.02279 |issn=1664-3224 |pmc=6180207 |pmid=30337927 |doi-access=free }}</ref> with a [[sex ratio]] of at least 9:1 female to male.<ref name="pmid20347176"/> The reasons for this disparity are unclear, but may involve the expression of sex hormones such as [[estrogen]], which impact immune system response.<ref name=":1" />

<!-- Society and culture -->
The condition has been recognised since at least 1851, and was named "primary biliary cirrhosis" in 1949.<ref name=DNS>{{cite journal | vauthors = Dauphinee JA, Sinclair JC | title = Primary biliary cirrhosis | journal = Canadian Medical Association Journal | volume = 61 | issue = 1 | pages = 1–6 | date = July 1949 | pmid = 18153470 | pmc = 1591584 }}</ref> 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.<ref name="PBCFname">{{cite web|last1=PBC Foundation (UK)|title=PBC Name Change|url=https://www.pbcfoundation.org.uk/news/collettes-blog/pbc-name-change|access-date=27 January 2017}}</ref><ref name="pbcers.org">{{cite web |title=Primary Biliary Cirrhosis Name Change Initiative |url=http://pbcers.org/education/NameChange1-3.pdf |archive-url=https://web.archive.org/web/20150419025303/http://pbcers.org/education/NameChange1-3.pdf |archive-date=19 April 2015 |access-date=15 September 2022 |website=PBCers Organization}}</ref>
{{TOC limit|3}}