Primary sclerosing cholangitis 864489 224270260 2008-07-08T02:26:33Z 207.201.211.242 /* Screening */ {{Unreferenced|date=December 2007}} {{Infobox_Disease | Name = {{PAGENAME}} | Image = PSC.jpg | Caption = [[Cholangiogram]] of primary sclerosing cholangitis. | DiseasesDB = 10643 | ICD10 = {{ICD10|K|83|0|k|80}} | ICD9 = {{ICD9|576.1}} | ICDO = | OMIM = | MedlinePlus = 000285 | eMedicineSubj = med | eMedicineTopic = 3556 | MeshID = D015209 | }} '''Primary sclerosing cholangitis''' ('''PSC''') is a form of [[cholangitis]] due to an [[autoimmune]] reaction. A cholangitis is an [[inflammation]] of the [[bile duct]]s of the [[liver]]. Primary sclerosing cholangitis leads to [[cholestasis]] (blockage of [[bile]] transport to the gut). Blockage of the bile duct leads to accumulation of bile, which damages the liver, leading to [[jaundice]] and eventually causes [[liver failure]]. ==Signs and symptoms== * Tiredness (a non-specific symptom often present in liver disease) * Severe [[jaundice]] with intense [[itch]]ing * [[Malabsorption]] (especially of [[fat]]) and [[steatorrhea]], leading to decreased levels of the fat-soluble [[vitamin]]s, [[vitamin A|A]], [[vitamin D|D]], [[vitamin E|E]] and [[vitamin K|K]]. * Signs of [[cirrhosis]] * [[Ascending cholangitis]], or [[infection]] of the bile duct. ==Diagnosis== The diagnosis is by imaging of the bile duct, usually in the setting of [[endoscopic retrograde cholangiopancreatography]] (ERCP, [[endoscopy]] of the bile duct and [[pancreas]]), which shows characteristic changes ("beading") of the bile ducts. Another option is [[magnetic resonance cholangiopancreatography]] (MRCP), where [[magnetic resonance imaging]] is used to visualise the biliary tract. Other tests often done are a [[full blood count]], [[liver enzyme]]s, [[bilirubin]] levels (usually grossly elevated), [[renal function]], [[electrolyte]]s. [[Fecal fat]] determination is occasionally ordered when the symptoms of malabsorption are prominent. The differential diagnosis can include [[primary biliary cirrhosis]], drug induced [[cholestasis]], [[cholangiocarcinoma]], and [[HIV]]-associated cholangiopathy. ==Etiology== The cause(s) for PSC are unknown. It is often considered to be an [[autoimmunity|autoimmune disorder]]. PSC is associated with [[ulcerative colitis]]. It is assumed that these diseases share a common cause. Ulcerative colitis is a systemic disease that affects many areas of the body. PSC is often listed as a manifestation of ulcerative colitis outside the colon. PSC differs from these manifestations in that, unlike most other manifestations, PSC continues in spite of surgical removal of the colon.[http://www.acg.gi.org] This suggests that, while the cause of ulcerative colitis, and most of its manifestations, is in the colon, the cause of PSC is located outside the colon. ==Pathophysiology== Bile ducts, both intra- and extrahepatically (inside the liver and outside), are inflamed and develop scarring, obstructing the flow of bile. As bile assists in the enteric breakdown and absorption of fat, the absence of bile leads to fat malabsorption. The bile accumulates in the duct, leading to liver cell damage and liver failure. ==Epidemiology== It is more prevalent in men than in women. The disease normally starts from age 30 to 60, though may begin in childhood. PSC progresses slowly, so the [[disease]] can be active for a long time before it is noticed or diagnosed. ==Screening== PSC is associated with [[cholangiocarcinoma]], which are tumors involving the biliary tree. Screening for cholangiocarcinoma in patients with PSC is encouraged, but there is no general consensus on the modality and interval of choice. Perinuclear antineutrophil cytoplasmic antibodies, also called [[p-ANCA]] are often characteristic of PSC (and [[Ulcerative Colitis]]). ==Therapy== Standard treatment includes [[ursodiol]], a bile acid naturally produced by the liver, which has been shown to lower elevated liver enzyme numbers in people with PSC, but has not yet been proven effective at prolonging the life of the liver. Treatment also includes medication to relieve itching ([[antipruritic]]s) and bile acid sequesterants (cholestyramine), [[antibiotic]]s to treat infections, and [[vitamin]] supplements, as people with PSC are often deficient in [[vitamin A]], [[vitamin D]], and [[vitamin K]]. In some cases, ERCP, which may involve stenting of the common bile duct, may be necessary in order to open major blockages (dominant strictures). [[Liver transplantation]] (including live transplants whereby a portion of a living donor is given to the recipient) is an option if the liver begins to fail. == See also == * [[Bilirubin]] * [[Child-Pugh score]] * [[Cholangiocarcinoma]] * [[Liver function tests]] * [[Primary biliary cirrhosis]] * [[Alagille syndrome]] * [[Progressive familial intrahepatic cholestasis]] * [[Walter Payton]] (died of primary sclerosing cholangitis) == External links == * [http://www.psc-literature.org Additional Literature about PSC] * [http://www.pscpartners.org Link to PSC Partners, a nonprofit foundation for PSC patients and caregivers] * [http://www.psctrust.com/ PSC Trust is a medical research charity] * [http://www.liverfamilies.net Liver Families, a pediatric liver support group] * [http://www.pscfoundation.org/ The Morgan Foundation for the Study of PSC] {{Gastroenterology}} [[Category:Autoimmune diseases]] [[Category:Gastroenterology]] [[Category:Hepatology]] [[Category:Inflammations]] [[de:Primär sklerosierende Cholangitis]] [[es:Colangitis esclerosante primaria]] [[ja:原発性硬化性胆管炎]] [[pl:Pierwotne stwardniające zapalenie dróg żółciowych]] [[sv:Primär skleroserande kolangit]] [[et:Primaarne skleroseeriv kolangiit]]