Snapshot A 62-year-old man presents to his primary care physician complaining of constant itchings and yellow skin and eyes. On exam, there is no evidence of hepatomegaly, however, laboratory findings are of concern for elevated serum ALP and GGT. Introduction Chronic cholestatic syndrome characterized by fibrosing inflammation in the intrahepatic and extrahepatic bile duct onset usually insidious leads to narrowing and, eventually, obliteration of the bile ducts Cause of primary sclerosing cholangitis (PSC) is unknown PSC occurs most often in young men (< 50 years old) HLA-DR52a positive commonly associated with inflammatory bowel disease especially ulcerative colitis Presentation Symptoms pruritus jaundice some present with hepatosplenomegaly or features of cirrhosis Evaluation Labs ↑ serum ALP, GGT bilirubin ↑ with conjugated fraction > 50% typical of obstructive pattern no urine urobilinogen low urine urobilin Endoscopic retrograde cholangiography (ERCP) give the biliary tree an irregular beaded appearance Treatment Liver transplantation prior to liver failure is a possibility in selected cases Prognosis, Prevention, and Complications Prognosis is poor May result in liver failure, cirrhosis, cholangiocarcinoma