21-hydroxylase autoantibodies in adult patients with endocrine autoimmune diseases are highly specific for Addison's disease. Belgian Diabetes Registry

Clin Exp Immunol. 1997 Feb;107(2):341-6. doi: 10.1111/j.1365-2249.1997.262-ce1153.x.


The diagnostic specificity of recombinant 21-hydroxylase autoantibodies (21OH-Ab) for Addison's disease was tested in adult patients with either Graves' disease (GD), insulin-dependent diabetes mellitus (IDDM), or polyendocrinopathy, as well as in healthy controls. Using a radiobinding assay with in vitro translated recombinant human 21-hydroxylase, we found 21OH-Ab in 24/28 (86%) idiopathic Addison patients, and using an immunofluorescence assay we found adrenal cortex autoantibodies (ACA) in 12/28 (43%) patients (P = 0.002). All the 12 ACA-positive sera were also positive for 21OH-Ab and ACA were found in 11/15 (73%) patients with less than 15 years and in 1/13 (8%) patients with 15-38 years of disease duration (P = 0.002). 21OH-Ab were present in 3/92 (3%) patients with GD, in 1/180 (0.6%) with IDDM and in 0/106 healthy subjects. The 21OH-Ab-positive GD and IDDM patients were also positive for ACA. None of 17 patients with polyendocrinopathy, but without Addison's disease, had 21OH-Ab. None of the 180 Belgian IDDM patients had Addison' s disease or developed an adrenal insufficiency at follow up. In two out of three Graves patients, the presence of 21OH-Ab was associated with clinical and biochemical signs of adrenal insufficiency. Of the 89 21OH-Ab-negative patients with GD none had Addison's disease at the time of blood sampling, and 79 were followed up for 5.6-7.5 years and none developed clinical signs of adrenal insufficiency. We conclude that the presence of 21OH-Ab in patients with endocrine autoimmune diseases is highly specific for Addison's disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Addison Disease / epidemiology
  • Addison Disease / immunology*
  • Adrenal Cortex / immunology
  • Adult
  • Antibody Specificity
  • Autoantibodies / immunology
  • Belgium / epidemiology
  • Diabetes Mellitus, Type 1 / immunology
  • Graves Disease / immunology
  • Humans
  • Italy / epidemiology
  • Polyendocrinopathies, Autoimmune / epidemiology
  • Polyendocrinopathies, Autoimmune / immunology*
  • Predictive Value of Tests
  • Prevalence
  • Recombinant Proteins / immunology
  • Steroid 21-Hydroxylase / immunology*


  • Autoantibodies
  • Recombinant Proteins
  • Steroid 21-Hydroxylase