Snap Shot A 28-year-old male with a past medical history significant for ulcerative colitis presents with a gradual worsening fatigue associated with jaundice. Introduction A chronic cholestatic syndrome characterized by fibrosing inflammation in the intrahepatic and extrahepatic bile ducts leading to narrowing and, eventually, obliteration of the bile ducts leads to cirrhosis The cause of primary sclerosing cholangitis (PSC) is unknown. theoretic pathogens include toxins, infectious agents, and abnormalities in immune regulation. PSC occurs most often in: young men commonly associated with inflammatory bowel disease (especially ulcerative colitis) 7 to 10% of patients with PSC develop cholangiocarcinoma. optimal timing of transplantation to prevent this complication is unknown Presentation Symptoms/Physical exam the onset is usually insidious with gradual, progressive fatigue pruritus jaundice some present with hepatosplenomegaly or features of cirrhosis the terminal phase is characterized by decompensated cirrhosis portal hypertension ascites liver failure Evaluation Labs elevated serum alkaline phosphatase bilirubin elevation is variable PSC is most readily diagnosed by direct cholangiography (preferably ERCP) which shows multiple short strictures and saccular dilations involving intrahepatic and extrahepatic bile ducts give the biliary tree an irregular beaded appearance The diagnosis is also supported by liver biopsy which shows bile duct proliferation periductal fibrosis and inflammation loss of bile ducts PSC the mitochondrial antibody test is negative (unlike in primary biliary cirrhosis) As the disease progresses, fibrosis extends from the portal regions and eventually leads to biliary cirrhosis. Treatment Endoscopic balloon dilation of strictures may relieve symptoms Endoscopic stent (drain or tube) placement for major strictures may be a possibility Liver transplantation prior to liver failure is a possibility in selected cases Prognosis, Prevention, and Complications Prognosis is poor most persons survive just a few years after the symptoms develop Liver failure,cholangiocarcinoma