IgG4-related systemic disease (IRSD) is a recently described entity with protean manifestations. We describe a patient who developed inflammation and fibrosis in multiple organs over 20 years, sequentially involving his pancreas, bile ducts, gallbladder, submandibular and lacrimal glands, and kidneys. He had an elevated serum IgG4 level. Retrospective analysis of biopsies showed strongly positive tissue immunostaining for IgG4, confirming the diagnosis of IRSD. This case illustrates the natural history of partially treated IRSD and its varied clinical presentations. Early diagnosis and treatment is important, as the condition is highly steroid-responsive.
A 55-year-old man who abstained from drinking alcohol and was not taking any medications initially presented to a hepatobiliary surgeon in 1989 for recurrent abdominal pain and jaundice. On examination, he was icteric, with mild epigastric tenderness, but was afebrile and had no other signs of chronic liver disease. His serum amylase level was mildly elevated, and results of liver function tests (LFTs) showed abnormal levels of albumin (29 g/L; reference range [RR], 36–48 g/L), alkaline phosphatase (284 U/L; RR, 32–91 U/L), γ-glutamyltransferase (98 U/L; RR, < 38 U/L), alanine transaminase (50 U/L; RR, < 34 U/L) and bilirubin (102 μmol/L; RR, < 18 μmol/L).
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