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Primary sclerosing cholangitis
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==Management== No pharmacologic treatment has been approved by the U.S. [[Food and Drug Administration]] for PSC. Some experts recommend a trial of [[ursodeoxycholic acid]] (UDCA), a bile acid occurring naturally in small quantities in humans, as it has been shown to lower elevated liver enzyme numbers in patients with PSC and has proven effective in other cholestatic liver diseases. However, UDCA has yet to be shown to clearly lead to improved liver histology and survival.<ref name="ReferenceA" /><ref name="Lindor">{{cite journal | vauthors = Lindor KD, Kowdley KV, Luketic VA, Harrison ME, McCashland T, Befeler AS, Harnois D, Jorgensen R, Petz J, Keach J, Mooney J, Sargeant C, Braaten J, Bernard T, King D, Miceli E, Schmoll J, Hoskin T, Thapa P, Enders F | display-authors = 6 | title = High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis | journal = Hepatology | volume = 50 | issue = 3 | pages = 808β814 | date = September 2009 | pmid = 19585548 | pmc = 2758780 | doi = 10.1002/hep.23082 }}</ref> Guidelines from the [[American Association for the Study of Liver Diseases]] and the [[American College of Gastroenterology]] do not support the use of UDCA but guidelines from the [[European Association for the Study of the Liver]] do endorse the use of moderate doses (13β15 milligrams per kilogram) of UDCA for PSC.<ref name="Lazaridis2016" /><ref name="Chapman2010">{{cite journal | vauthors = Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ | title = Diagnosis and management of primary sclerosing cholangitis | journal = Hepatology | volume = 51 | issue = 2 | pages = 660β678 | date = February 2010 | pmid = 20101749 | doi = 10.1002/hep.23294 | s2cid = 35432726 | doi-access = }}</ref><ref name="Lindor2015">{{cite journal | vauthors = Lindor KD, Kowdley KV, Harrison ME | title = ACG Clinical Guideline: Primary Sclerosing Cholangitis | journal = The American Journal of Gastroenterology | volume = 110 | issue = 5 | pages = 646β59; quiz 660 | date = May 2015 | pmid = 25869391 | doi = 10.1038/ajg.2015.112 | s2cid = 41646016 | doi-access = free }}</ref><ref name="EASL2009">{{cite journal | title = EASL Clinical Practice Guidelines: management of cholestatic liver diseases | journal = Journal of Hepatology | volume = 51 | issue = 2 | pages = 237β267 | date = August 2009 | pmid = 19501929 | doi = 10.1016/j.jhep.2009.04.009 | doi-access = free | author1 = European Association for the Study of the Liver }}</ref> Supportive treatment for PSC symptoms is the cornerstone of management. These therapies are aimed at relieving symptoms such as itching with [[antipruritic]]s (e.g. [[bile acid sequestrant]]s such as [[cholestyramine]]); [[antibiotic]]s to treat episodes of [[ascending cholangitis]]; and vitamin supplements, as people with PSC are often deficient in fat-soluble vitamins (A, D, E, and K).<ref>{{cite journal | title = EASL Clinical Practice Guidelines: management of cholestatic liver diseases | journal = Journal of Hepatology | volume = 51 | issue = 2 | pages = 237β267 | date = August 2009 | pmid = 19501929 | doi = 10.1016/j.jhep.2009.04.009 | doi-access = free | author1 = European Association for the Study of the Liver }}</ref> ERCP and specialized techniques may also be needed to help distinguish between a benign PSC stricture and a bile-duct cancer (cholangiocarcinoma).<ref>{{cite journal | vauthors = Tabibian JH, Visrodia KH, Levy MJ, Gostout CJ | title = Advanced endoscopic imaging of indeterminate biliary strictures | journal = World Journal of Gastrointestinal Endoscopy | volume = 7 | issue = 18 | pages = 1268β1278 | date = December 2015 | pmid = 26675379 | pmc = 4673389 | doi = 10.4253/wjge.v7.i18.1268 | doi-access = free }}</ref> Liver transplantation is the only proven long-term treatment of PSC. Indications for transplantation include recurrent bacterial ascending cholangitis, decompensated cirrhosis, [[hepatocellular carcinoma]], hilar cholangiocarcinoma, and complications of portal hypertension. Not all patients are candidates for liver transplantation, and some experience disease recurrence afterward.<ref name="ReferenceB" /> The reasons why some patients develop recurrent PSC remains largely obscure, but surprisingly, those without recurrence of disease (hence protected from recurrence) are characterized by an increased presence of the potentially pathogenic '' [[Shigella]]'' species.<ref>{{cite journal | vauthors = Visseren T, Fuhler GM, Erler NS, Nossent YR, Metselaar HJ, IJzermans JN, Darwish Murad S, Peppelenbosch MP | display-authors = 6 | title = Recurrence of primary sclerosing cholangitis after liver transplantation is associated with specific changes in the gut microbiome pretransplant β a pilot study | journal = Transplant International | volume = 33 | issue = 11 | pages = 1424β1436 | date = November 2020 | pmid = 33617049 | pmc = 7689804 | doi = 10.1111/tri.13692 }}</ref>
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