Editing Primary sclerosing cholangitis (section)

{{Short description|Inflammatory disease of the bile ducts}}
{{Infobox medical condition (new)
| name = Primary sclerosing cholangitis
| synonyms = 
| image = Cholangiogram of primary sclerosing cholangitis.jpg
| caption = [[Cholangiogram]] of primary sclerosing cholangitis
| pronounce = 
| field = [[Gastroenterology]]
| symptoms = Typically no symptoms; abdominal pain, fatigue, fever, [[jaundice]]
| complications = [[Cholangiocarcinoma]]
| onset = 
| duration = Long term and progressive
| types = 
| causes = Unknown; most likely [[Autoimmune disease|autoimmune]]
| risks = [[Inflammatory bowel disease]], pre-existing liver disease
| diagnosis = Radiological imaging
| differential = Secondary causes of sclerosing cholangitis
| prevention = 
| treatment = Symptomatic
| medication = 
| prognosis = 
| frequency = 
| deaths = 
}}
<!-- Definition and symptoms -->

'''Primary sclerosing cholangitis''' ('''PSC''') is a long-term progressive disease of the [[liver]] and [[gallbladder]] characterized by [[inflammation]] and scarring of the [[bile duct]]s, which normally allow [[bile]] to drain from the gallbladder. Affected individuals may have no symptoms or may experience signs and symptoms of liver disease, such as [[jaundice]], [[pruritus|itching]], and abdominal pain.

<!-- Cause and Pathophysiology -->
The bile duct scarring that occurs in PSC narrows the ducts of the [[Biliary tract|biliary tree]] and impedes the flow of bile to the [[duodenum]]. Eventually, it can lead to [[cirrhosis]] of the liver and [[liver failure]]. PSC increases the risk of various cancers, including [[hepatocellular carcinoma|liver cancer]], [[gallbladder carcinoma]], [[colorectal cancer]], and [[cholangiocarcinoma]].<ref name="Lazaridis2016">{{cite journal | vauthors = Lazaridis KN, LaRusso NF | title = Primary Sclerosing Cholangitis | journal = The New England Journal of Medicine | volume = 375 | issue = 12 | pages = 1161–1170 | date = September 2016 | pmid = 27653566 | pmc = 5553912 | doi = 10.1056/NEJMra1506330 | type = Review }}</ref><ref name="Folseraas2016">{{cite journal | vauthors = Folseraas T, Boberg KM | title = Cancer Risk and Surveillance in Primary Sclerosing Cholangitis | journal = Clinics in Liver Disease | volume = 20 | issue = 1 | pages = 79–98 | date = February 2016 | pmid = 26593292 | doi = 10.1016/j.cld.2015.08.014 }}</ref>  The underlying cause of PSC is unknown. Genetic susceptibility, [[autoimmunity|immune system dysfunction]], and [[dysbiosis|abnormal composition of the gut flora]] may play a role.<ref name="Kummen2013" /><ref name="Charatchar">{{cite journal | vauthors = Charatcharoenwitthaya P, Lindor KD | title = Primary sclerosing cholangitis: diagnosis and management | journal = Current Gastroenterology Reports | volume = 8 | issue = 1 | pages = 75–82 | date = February 2006 | pmid = 16510038 | doi = 10.1007/s11894-006-0067-8 | s2cid = 37474299 }}</ref> This is further suggested by the observation that around 75% of individuals with PSC also have [[inflammatory bowel disease]] (IBD), most often [[ulcerative colitis]].<ref>{{cite book| vauthors = Sleisenger MH |title=Sleisenger and Fordtran's gastrointestinal and liver disease: pathophysiology, diagnosis, management |year=2006 |publisher=Saunders |location=Philadelphia |edition=8th}}</ref>

<!-- Prevention and Treatment -->
No effective medical treatment for primary sclerosing cholangitis is known. Its most definitive treatment is a [[liver transplantation|liver transplant]],<ref name="Lazaridis2016" /> but disease recurrence can occur in 25–30% of cases.<ref name="Melb2022">{{cite journal |last1=Tan |first1=Natassia |last2=Ngu |first2=N. |last3=Lee |first3=T. |last4=Abrahams |first4=T. |last5=Pandya |first5=K. |last6=Freeman |first6=E. |last7=Hannah |first7=N. |last8=Gazelakis |first8=K. |last9=Madden |first9=R. |last10=Lynch |first10=K. |last11=Valaydon |first11=Z. |last12=Sood |first12=S. |last13=Dev |first13=A. |last14=Bell |first14=S. |last15=Thompson |first15=A. |last16=Ding |first16=J. |last17=Nicoll |first17=A. |last18=Liu |first18=K. |last19=Gow |first19=P. |last20=Lubel |first20=J. |last21=Kemp |first21=W. |last22=Roberts |first22=S. |last23=Majeed |first23=A. |title=Epidemiology and outcomes of primary sclerosing cholangitis: an Australian multicentre retrospective cohort study |journal=Hepatology International |date= June 2022 |volume=16 |issue=5 |pages=1094–1104 |doi=10.1007/s12072-022-10356-1 |pmid=35657479 |pmc=9525417 }}</ref> For patients unable or unwilling to receive a transplant, therapy primarily focuses on relieving symptoms, rather than stopping disease progression. If the sclerosing cholangitis is a ''secondary'' effect of a different disease, treatment is directed towards the underlying cause.<ref name=":2">{{Cite journal |last1=Karlsen |first1=Tom H. |last2=Folseraas |first2=Trine |last3=Thorburn |first3=Douglas |last4=Vesterhus |first4=Mette |date=December 2017 |title=Primary sclerosing cholangitis – a comprehensive review |url=https://linkinghub.elsevier.com/retrieve/pii/S0168827817321967 |journal=Journal of Hepatology |language=en |volume=67 |issue=6 |pages=1298–1323 |doi=10.1016/j.jhep.2017.07.022|pmid=28802875 |doi-access=free }}</ref>

<!-- Epidemiology -->
PSC is a rare disease and most commonly affects people with IBD.<ref name="Folseraas2016"/> About 3.0 to 7.5% of people with ulcerative colitis have PSC, and 80% of people with PSC have some form of IBD.<ref name="Kummen2013">{{cite journal | vauthors = Kummen M, Schrumpf E, Boberg KM | title = Liver abnormalities in bowel diseases | journal = Best Practice & Research. Clinical Gastroenterology | volume = 27 | issue = 4 | pages = 531–542 | date = August 2013 | pmid = 24090940 | doi = 10.1016/j.bpg.2013.06.013 }}</ref> Diagnosis usually occurs in people in their 30s or 40s.<ref name="Kummen2013"/> Individuals of Northern European ancestry are affected more often than people of Southern European or Asian descent.<ref name="Folseraas2016"/> Men are affected more often than women.<ref name="Williamson2015">{{cite journal | vauthors = Williamson KD, Chapman RW | title = Primary sclerosing cholangitis: a clinical update | journal = British Medical Bulletin | volume = 114 | issue = 1 | pages = 53–64 | date = June 2015 | pmid = 25981516 | doi = 10.1093/bmb/ldv019 | doi-access = free }}</ref> The disease was initially described in the mid-1800s, but was not fully characterized until the 1970s with the advent of improved medical-imaging techniques such as [[endoscopic retrograde cholangiopancreatography]].<ref name="Williamson2015"/>