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

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 ?
In classical primary sclerosing cholangitis (PSC), the stenoses would have been shorter, giving the cholangiogram a more beaded appearance.
Both MUC5AC and MUC6 are expressed in biliary epithelium in inflammatory conditions, such as primary sclerosing cholangitis. (6, 15)
* The report provides a snapshot of the global therapeutic landscape of Primary Sclerosing Cholangitis
Risk factors of cholangiocarcinogenesis, after Ghouri et al [2] Primary sclerosing cholangitis Hepatolithiasis Liver flukes--Opistorchis vierrini and Clonorchis sinensis Caroli's disease Congenital hepatic fibrosis Viral hepatitis B and C infection Liver cirrhosis Obesity and diabetes Chemical compounds--dioxin, thorotrast Choledocal cysts
She had been enduring a chronic liver condition that compromises the immune system called primary sclerosing cholangitis since she was 19 but had managed the condition throughout her tennis career through regular blood tests and medication.
The daughter of Sergei Baltacha, who played professional football for Ipswich Town and Inverness Caledonian Thistle, she was diagnosed with primary sclerosing cholangitis, a chronic liver condition which compromises the immune system, aged 19.
Katrina Angeles was diagnosed with a chronic liver disease called primary sclerosing cholangitis cirrhosis.
Then, in 1999, after a routine blood test showed I had elevated liver enzymes, a series of tests confirmed I have a rare autoimmune disease called primary sclerosing cholangitis (PSC).
Primary Sclerosing Cholangitis. Primary sclerosing cholangitis (PSC) is a fibrosclerotic disease of the bile ducts, with diffuse structuring of the intrahepatic and extrahepatic biliary tree [58, 59].
Peter Cosgrove, 49, decided to take on the fitness challenge after his colleague, Ericka Oates' son Callum, 13, was diagnosed with the rare liver condition, primary sclerosing cholangitis.
Torchbearer WILLIAM WILLIAMS, 68, is an active supporter of various local and national charities, despite being a sufferer of the "no-known-cause, no-known-cure", chronic progressive disease Primary Sclerosing Cholangitis (PSC).

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