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 IgG₄-associated sclerosing cholangitis affecting the extrahepatic biliary ducts and mimicking primary sclerosing cholangitis and cholangiocarcinoma. Management: Corticosteroids and immunomodulatory therapy.