Autoantibodies to steroidogenic enzymes in patients with premature ovarian failure with and without Addison's disease

Eur J Endocrinol. 2003 May;148(5):565-70. doi: 10.1530/eje.0.1480565.

Abstract

Design: Adrenal cortex autoantibodies (ACA), steroid-producing cell autoantibodies (StCA) and autoantibodies (Abs) to steroidogenic enzymes in three groups of patients with premature ovarian failure (POF), 15 with autoimmune Addison's disease (AD), 26 with non-adrenal autoimmune diseases and 31 with isolated POF, have been assessed.

Methods: ACA and StCA were measured using an immunofluorescence technique. Abs to 21-hydroxylase (21-OH), to 17alpha-hydroxylase (17alpha-OH) and to cytochrome P450 side-chain cleavage (P450scc) were measured using an immunoprecipitation assay.

Results: Seventy-three percent of patients with POF and AD were positive for StCA, 93% for 17alpha-OH and/or P450scc Abs, 93% for ACA and 100% for 21-OH Abs. Among patients with POF and non-adrenal autoimmune diseases, 8% were positive for StCA, 12% for 17alpha-OH and/or P450scc Abs, and 8% and 12% for ACA and 21-OH Abs respectively. StCA, 17alpha-OH and/or P450scc Abs were all found in 10% of patients with isolated POF, and 13% had ACA and 21-OH Abs. All StCA-, 17alpha-OH- and/or P450scc Abs-positive patients were also positive for ACA and 21-OH Abs. Two patients with isolated POF who were ACA and 21-OH Ab positive developed AD 3 and 5 Years after the onset of POF.

Conclusion: This study has shown that, when POF is associated with AD, StCA, 17alpha-OH and/or P450scc Abs are present in the majority of patients, while in the other two groups these Abs are detectable in a much lower proportion of patients. Measurement of ACA/21-OH Abs in some patients with POF may be important in identifying patients at risk of developing overt AD.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Addison Disease / complications
  • Addison Disease / immunology*
  • Adrenal Cortex / immunology
  • Adult
  • Autoantibodies / analysis*
  • Autoimmune Diseases / immunology
  • Cholesterol Side-Chain Cleavage Enzyme / immunology
  • Enzymes / immunology*
  • Enzymes / metabolism*
  • Female
  • Humans
  • Primary Ovarian Insufficiency / complications
  • Primary Ovarian Insufficiency / immunology*
  • Steroid 17-alpha-Hydroxylase / immunology
  • Steroids / biosynthesis*

Substances

  • Autoantibodies
  • Enzymes
  • Steroids
  • Steroid 17-alpha-Hydroxylase
  • Cholesterol Side-Chain Cleavage Enzyme