An 80-year-old woman was admitted due to liver dysfunction. She had been treated for type 2 diabetes mellitus by oral anti-diabetic medications. Laboratory examinations findings were as follows: AST 96 IU/L, ALT 134 IU/L, γ -GTP 823 IU/L, fasting serum glucose 204 mg/dL, HbA1c 8.3 %, C-peptide 2.46 ng/mL, anti-GAD antibody 55.8 U/mL, and IgG4 704 mg/dL. Imaging showed marked dilation of bile ducts without swelling of the pancreas or narrowing of the pancreatic ducts. The histological findings of a liver biopsy were compatible with IgG4-related sclerosing cholangitis. Both steroid therapy and insulin therapy were started and led to the amelioration of the liver function and insulin secretion as well as a reduction in anti-GAD antibody. We herein report a case of IgG4-related sclerosing cholangitis associated with diabetes mellitus with positivity for anti-GAD antibody, a situation that is rare to our knowledge.
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