primary sclerosing cholangitis


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primary sclerosing cholangitis

idiopathic chronic hepatobiliary disease characterized by diffuse inflammation and fibrosis of the extrahepatic biliary system resulting in patchy, irregular stricturing of the bile ducts. Progressive bile duct obliteration to cirrhosis, hepatic failure, and bile duct cancer may occur in up to 50% of patients. Frequently associated with inflammatory bowel disease.

primary sclerosing cholangitis

a progressive chronic fibrosing inflammation of the bile ducts of unknown cause, occurring most commonly in young men and frequently in association with chronic ulcerative colitis. It also occurs as a complication of HIV infection. See also AIDS cholangiopathy.

primary sclerosing cholangitis

Sclerosing cholangitis A chronic idiopathic progressive liver disease characterized by inflammation, necrosis, fibrosis and obliteration of intrahepatic and extrahepatic bile ducts, resulting in cholestasis, cirrhosis, portal HTN, liver failure, often associated with other autoimmune diseases–eg, Crohn's disease, ulcerative colitis, Addison's disease Epidemiology PSC is uncommon–1 to 6/105, US; it is the 4th most common indication for liver transplant; 75% of Pts have IBD; 70% of Pts are ♂, average age 39 Clinical Often asymptomatic until end-stage liver disease; as albumin ↓ and BR ↑, pruritus, fatigue, jaundice, and weight loss dominate clinical picture, ± accompanied by fever, chills, night sweats, right upper quadrant pain Imaging Multiple zones of narrowing and dilatation– “beading” of bile duct by ERCP and transhepatic cholangiography Lab ↑ Alk phos, ↑ aminotransferase, hypergammaglobulinemia, ↑ IgM Management Azacytidine may ↓ need for transfusion, iron chelation–early use of deferiprone, corticosteroids, penicillamine, MTX; ursodiol is useless Prognosis Median survival after diagnosis, 12 yrs. See Primary biliary cirrhosis.

Primary sclerosing cholangitis

A chronic disease in which it is believed that the immune system fails to recognize the cells that compose the bile ducts as part of the same body, and attempts to destroy them. It is not clear what exactly causes the disease, but it is frequently associated with another inflammatory disease of the digestive tract, ulcerative colitis. The inflammation of the ducts eventually produces formation of scar tissue, causing multiple areas of narrowing (strictures) that block bile flow and lead to bacterial infection. Liver transplant gives the best chance for long-term survival.
Mentioned in: Cholangitis
References in periodicals archive ?
The few worthwhile treatments for primary sclerosing cholangitis have been shown to improve measures of liver biochemistry--but not disease progression, Dr.
Taniai et al (19) reported that functional point mutations in the p16INK4a promoter region likely contribute to the initiation/progression of cholangiocarcinoma in primary sclerosing cholangitis.
MR cholangiopancreatography also continues to gain acceptance as an initial modality by which patients with suspected primary sclerosing cholangitis (PSC) are assessed (Figure 9).
31) reported loss of antigenic recognition after DNA digestion of neutrophils for UC P-ANCAs but not for P-ANCAs of primary sclerosing cholangitis and autoimmune hepatitis.
Liver transplants are used for a range of diseases including hepatitis, primary sclerosing cholangitis and biliary atresia.
Miguel Zavala 48, a factory worker and 30-year member of the Holy Family Catholic Church in Waukegan, was diagnosed 3 years ago with primary sclerosing cholangitis (PSC), a rare disease of the bile ducts and the same disorder that claimed the life of Football Hall of Fame running back Walter Payton.
In 1993, he was diagnosed with a rare liver disease called primary sclerosing cholangitis, the same illness that killed Chicago Bears great Walter Payton.
The recipient was suffering from primary sclerosing cholangitis, a chronic inflammation and fibrosis of all bile ducts.
Payton had suffered from primary sclerosing cholangitis, a rare liver disease that could only be cured by a transplant.
Primary sclerosing cholangitis is a disease in which the bile ducts inside and outside the liver become narrower due to inflammation and scarring.
It also reviews key players involved in the therapeutic development for Primary Sclerosing Cholangitis.
NGM282 is currently in development for the treatment of primary biliary cirrhosis, recently renamed primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and nonalcoholic steatohepatitis (NASH).

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