Autoimmune polyglandular syndrome III in a patient with idiopathic portal hypertension

Intern Med. 2013;52(12):1375-8. doi: 10.2169/internalmedicine.52.8796.

Abstract

A 42-year-old woman with a history of idiopathic portal hypertension (IPH) developed type 1A diabetes and was found to have chronic thyroiditis. The concurrence of IPH and type 1A diabetes has been previously reported in only one case. This is the second known case, and our patient was classified as having autoimmune polyglandular syndrome (APS) III. The patient's HLA DR and DQ alleles were determined to be susceptible to autoimmune thyroid diseases but resistant to type 1A diabetes.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Diabetes Mellitus, Type 1 / complications
  • Diabetes Mellitus, Type 1 / genetics
  • Diabetes Mellitus, Type 1 / immunology
  • Female
  • HLA-DQ Antigens / genetics
  • HLA-DR Antigens / genetics
  • Humans
  • Hypertension, Portal / complications*
  • Hypertension, Portal / genetics
  • Hypertension, Portal / immunology
  • Idiopathic Noncirrhotic Portal Hypertension
  • Liver Cirrhosis / complications*
  • Liver Cirrhosis / genetics
  • Liver Cirrhosis / immunology
  • Pancytopenia / complications*
  • Pancytopenia / genetics
  • Pancytopenia / immunology
  • Polyendocrinopathies, Autoimmune / complications*
  • Polyendocrinopathies, Autoimmune / genetics
  • Polyendocrinopathies, Autoimmune / immunology
  • Splenomegaly / complications*
  • Splenomegaly / genetics
  • Splenomegaly / immunology

Substances

  • HLA-DQ Antigens
  • HLA-DR Antigens