A case of IgG4-associated cholangitis and autoimmune pancreatitis responsive to corticosteroids

Background 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. Investigations Biochemical and serologic studies, CT scan, abdominal ultrasound, endoscopic ultrasound, endoscopic retrograde cholangiopancreatography, biliary cytology, pancreas needle biopsies and immunohistochemical stainings. Diagnosis Autoimmune pancreatitis with IgG(4)-associated sclerosing cholangitis affecting the extrahepatic biliary ducts and mimicking primary sclerosing cholangitis and cholangiocarcinoma. Management Corticosteroids and immunomodulatory therapy.