Abstract

A 72-year-old male presented to the emergency department with epigastric pain, anorexia and progressive jaundice of 1 week's duration. He had no prior history of gastrointestinal illness, diabetes or cancer. He did not smoke or consume alcohol. He did have a family history of colon and bone cancer. Biochemical and serologic studies, CT scan, abdominal ultrasound, endoscopic ultrasound, endoscopic retrograde cholangiopancreatography, biliary cytology, pancreas needle biopsies and immunohistochemical stainings. Autoimmune pancreatitis with IgG(4)-associated sclerosing cholangitis affecting the extrahepatic biliary ducts and mimicking primary sclerosing cholangitis and cholangiocarcinoma. Corticosteroids and immunomodulatory therapy.

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