The patient with unexplained elevated serum liver enzymes.

The pattern of elevated serum liver enzymes in symptomatic or asymptomatic patients allows for an initial classification of liver diseases into cholestatic or hepatocellular diseases. A female patient with extrahepatic cholestasis due to segmental bile duct strictures and a localized mass lesion within the pancreas is presented. Although many diagnostic procedures were performed in this case the diagnosis was not obtained before surgical laparotomy was initiated with bioptic sampling from bile ducts, lymph nodes and pancreatic tissue. Microscopic examination of the specimen revealed extensive biliary and pancreatic scarring together with periductal infiltrates composed of lymphocytes and plasma cells consistent with sclerosing cholangitis in systemic autoimmune pancreatitis. The patient completely recovered upon treatment with prednisone and azathioprine. The difficult approach to the final diagnosis is discussed in light of established and modern diagnostic tools.