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.