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Clinical characteristics of polyglandular autoimmune syndromes in pediatric age: an observational study

Bruno Bouça1, Andreia Nogueira2, Joana Caetano3

  • 1Endocrinology, Diabetes and Metabolism Department, Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal.

Journal of Pediatric Endocrinology & Metabolism : JPEM
|February 16, 2022
PubMed

Insights

Polyglandular autoimmune syndromes (PAS) are often underestimated in children. This study found PAS type 3 most common in pediatric patients with autoimmune diseases like type 1 diabetes and thyroid disease.

Area of Science:

  • Endocrinology
  • Immunology
  • Pediatrics

Background:

  • Polyglandular autoimmune syndromes (PAS) involve multiple autoimmune diseases (AID).
  • While PAS type 1 is common in childhood, other PAS types are less studied in pediatric populations.
  • The prevalence of various PAS types in children may be underestimated.

Purpose of the Study:

  • To determine the prevalence of different PAS types in a pediatric cohort.
  • To describe and classify PAS manifestations in children with specific endocrine autoimmune diseases.
  • To compare the age of onset of autoimmune diseases in children with and without PAS.

Main Methods:

  • A 10-year retrospective study of 879 children diagnosed with Diabetes mellitus type 1 (DM1), autoimmune thyroid disease (AITD), or Addison's disease (AD).
  • Evaluation for the presence of multiple autoimmune diseases to diagnose and classify PAS types.
  • Statistical analysis to compare disease onset age between children with PAS and monoglandular conditions.

Main Results:

  • 35 children were diagnosed with PAS, with PAS type 3 being the most prevalent (65.7%).
  • Autoimmune thyroid disease (AITD) was the most frequently observed autoimmune disease (74.3%) among PAS patients.
  • PAS type 1 was not diagnosed in this cohort.
  • Children with PAS presented with DM1 and AITD at a significantly younger age compared to those with single autoimmune conditions.

Conclusions:

  • This study provides novel insights into the prevalence of diverse PAS types within a pediatric endocrine patient group.
  • Early detection of autoantibodies is crucial for timely PAS diagnosis, especially in individuals with Addison's disease.
  • Lifelong screening for PAS is recommended for pediatric patients diagnosed with Addison's disease.
Abstract

Keywords:
Addison’ diseaseautoimmune thyroid diseasepolyglandular autoimmune syndromestype 1 diabetes mellitus

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