Autoimmune pancreatitis is characterised by diffuse enlargement of pancreas, narrowing of pancreatic duct, lymphoplasmacytic infiltrations and fibrosis. The disease is responsive to corticosteroid. We report the case of a 32-year-old male who presented with unilateral exophthalmos and obstructive jaundice secondary to pancreatic head mass and biliary tract stricture. Serum immunoglobulin G level was raised with a very high immunoglobulin G4 subclass. Ophthalmological imaging revealed unilateral thickening of extraocular muscles. The patient responded well to corticosteroid with resolution of biliary strictures, pancreatic head mass and exophthalmos.
Keywords: Autoimmune pancreatitis; IgG4-related systemic disease; Exophthalmos.