Prevalence of other autoimmune diseases in polyglandular autoimmune syndromes type II and III

J Endocrinol Invest. 2020 Sep;43(9):1-9. doi: 10.1007/s40618-020-01229-1. Epub 2020 Aug 17.


Purpose: Polyglandular autoimmune syndromes (PAS) are complex, heterogeneous disorders in which various autoimmune diseases can occur, affecting both endocrine and non-endocrine organs. In this meta-analysis, the prevalence of associated autoimmune disorders was investigated in PAS II and III.

Methods: A comprehensive search in MEDLINE and Embase databases identified 479 studies with the keywords of PAS II and PAS III. 18 records containing a total of 1312 patients fulfilled our inclusion criteria (original studies reporting at least 10 cases and containing the combination of other autoimmune disorders) and were selected for further analysis. A meta-analysis of prevalence was performed using the random-effects model with the calculation of 95% confidence intervals (CI). Results of each meta-analysis were displayed graphically using forest plots.

Results: Distinction between PAS II and PAS III was made in 842 cases, of which 177 and 665 were PAS II and III (21.1 vs 78.9%), respectively. The prevalence of Hashimoto's thyroiditis was significantly higher than that of Graves's disease (39% [95% CI 17-65%] vs. 4% [95% CI 0-10%], respectively; p = 0.001). In PAS II, Addison's disease (AD) coexisted with AITDs, T1DM or the combination of these conditions in 65, 18 and 10% of cases, respectively. In addition, one other endocrine and five non-endocrine organ-specific autoimmune disorders were reported. In PAS III, two other autoimmune endocrinopathies, six non-endocrine organ-specific, and four systemic autoimmune disorders were found in combination with AITDs.

Conclusions: AITDs, T1DM and AD are the most common combinations in PAS, thus screening for these conditions seems to be reasonable.

Keywords: Addison’s disease; Autoimmune thyroid disease; Diabetes mellitus; Meta-analysis; Polyglandular autoimmune syndrome.

Publication types

  • Meta-Analysis
  • Systematic Review

MeSH terms

  • Addison Disease / complications
  • Addison Disease / epidemiology
  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / epidemiology*
  • Child
  • Child, Preschool
  • Comorbidity
  • Diabetes Mellitus, Type 1 / complications
  • Diabetes Mellitus, Type 1 / epidemiology
  • Female
  • Graves Disease / complications
  • Graves Disease / epidemiology
  • Hashimoto Disease / complications
  • Hashimoto Disease / epidemiology
  • Humans
  • Male
  • Middle Aged
  • Polyendocrinopathies, Autoimmune / complications
  • Polyendocrinopathies, Autoimmune / epidemiology*
  • Polyendocrinopathies, Autoimmune / immunology
  • Prevalence
  • Young Adult