Primary Sclerosing Cholangitis (PSC)

Overview


Plain-Language Overview

Primary Sclerosing Cholangitis (PSC) is a chronic disease that affects the bile ducts, which are tubes that carry bile from the liver to the small intestine. This condition causes inflammation and scarring of these ducts, leading to blockages that interfere with the normal flow of bile. Bile is important for digesting fats and removing waste from the body. Over time, the damage can cause serious liver problems, including liver failure. People with PSC often experience symptoms like fatigue, itching, and jaundice, which is yellowing of the skin and eyes. PSC is also linked to other diseases, especially inflammatory bowel disease (IBD). Managing PSC involves monitoring liver health and addressing complications as they arise.

Clinical Definition

Primary Sclerosing Cholangitis (PSC) is a chronic, progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricturing of the intrahepatic and extrahepatic bile ducts. The exact etiology is unknown but is believed to involve an immune-mediated mechanism often associated with inflammatory bowel disease, particularly ulcerative colitis. The hallmark pathological feature is concentric periductal fibrosis leading to multifocal bile duct strictures and eventual biliary cirrhosis. Clinically, PSC presents with cholestasis, recurrent bacterial cholangitis, and progressive liver dysfunction. It significantly increases the risk of cholangiocarcinoma and requires lifelong monitoring. Diagnosis is supported by characteristic cholangiographic findings and exclusion of secondary causes of sclerosing cholangitis.

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Clinical Presentation


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Pathophysiology


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