2011 Volume 50 Issue 8 Pages 887-891
An autoimmune pancreatitis (AIP) patient with metachronous and multiple extrapancreatic lesions is reported. Initial symptoms were proptosis, oculomotor deficits, and a visual field deficit of the left eye, and swelling of bilateral lacrimal glands. Swelling of the right salivary gland and elevated serum levels of hepatobiliary enzymes were detected. AIP associated with IgG4-related orbital pseudotumor, IgG4-related sclerosing dacryoadenitis and sialadenitis, and IgG4-related sclerosing cholangitis was diagnosed. All symptoms and lesions improved with steroid therapy. Although an orbital pseudotumor is a rare extrapancreatic lesion of AIP, we should know that AIP patients may describe unusual symptoms such as abnormal visual field.