Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic fibroinflammatory cholangiopathy affecting the intrahepatic ducts, extrahepatic ducts, or both. Inflammation scars the duct walls and creates multiple irregular narrowings. Bile then moves poorly through the biliary tree, producing a cholestatic liver-test pattern and gradually causing biliary fibrosis, cirrhosis, and possible liver failure. The disease is frequently associated with inflammatory bowel disease, particularly ulcerative colitis. Colitis may be present without diarrhea or rectal bleeding, so a patient can have PSC-associated bowel disease before noticing intestinal symptoms. The combination also increases colorectal cancer risk and changes the required colonoscopy schedule. The damaged, narrowed ducts are vulnerable to bacterial infection. Bacteria can ascend into stagnant bile, causing acute bacterial cholangitis. Chronic cholestasis also contributes to pruritus, malabsorption of fat-soluble vitamins, and metabolic bone disease. As cirrhosis develops, portal hypertension may produce ascites, splenomegaly, varices, and gastrointestinal bleeding. PSC also carries important hepatobiliary cancer risks, especially cholangiocarcinoma and gallbladder carcinoma. A new change in the patient's pattern matters: worsening jaundice, rapidly increasing liver tests, progressive duct dilation, unexplained weight loss, or rapidly worsening pruritus can indicate...