Primary biliary cirrhosis and type II autoimmune polyglandular syndrome.
A 45-year-old female was diagnosed with Hashimoto's thyroiditis in 1976 and Addison's disease in 1979. At that time, her antimitochondrial antibody (AMA) level was elevated at 1:32. She subsequently developed premature ovarian failure and type I diabetes mellitus. In 1996, she became jaundiced with a cholestatic enzyme pattern. AMA was positive at a titre of 1:256. A liver biopsy confirmed the diagnosis of primary biliary cirrhosis (PBC). She underwent a liver transplantation in January 1998. This is the first report of PBC in association with type II autoimmune polyglandular syndrome. The association of PBC with other organ-specific autoimmune diseases supports an immune-mediated pathogenesis and may have implications in further studies of PBC.
Borgaonkar
M R
MR
Department of Medicine, McMaster University, Hamilton, Ontario. borgaonk@FHS.McMaster.ca
Morgan
D G
DG
D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008875:Middle Aged; D016884:Polyendocrinopathies, Autoimmune / Q000097:blood / Q000150:complications*