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Related Experiment Video

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Juvenile polyautoimmunity in a rheumatology setting.

Clara Malagón1, Maria Del Pilar Gomez2, Catalina Mosquera1

  • 1Postgraduate Pediatric Rheumatology Program, Universidad El Bosque, Bogota, Colombia; Grupo de Reumatología e Inmunología Pediátrica (GRIP), Colombia.

Autoimmunity Reviews
|February 18, 2019
PubMed
Summary

Overt polyautoimmunity (PolyA) in children involves multiple autoimmune diseases. This study identified common disease patterns and familial links, aiding early diagnosis in pediatric rheumatology. Keywords: polyautoimmunity, autoimmune diseases, pediatric rheumatology, early diagnosis.

Keywords:
Autoimmune diseasesAutoimmune tautologyJuvenile idiopathic arthritisJuvenile patientsPolyautoimmunitySystemic lupus erythematous

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Area of Science:

  • Pediatric Rheumatology
  • Immunology
  • Genetics

Background:

  • Overt polyautoimmunity (PolyA) is defined as the clinical manifestation of more than one distinct autoimmune disease in a single patient.
  • Understanding juvenile PolyA is crucial for timely diagnosis and management in pediatric rheumatology settings.

Purpose of the Study:

  • To characterize juvenile PolyA, including its chronological aspects, index diseases, familial aggregation, and disease clustering patterns.
  • To provide insights that can assist physicians in the early diagnosis of PolyA in pediatric patients.

Main Methods:

  • A cross-sectional, multicenter study involving 313 children diagnosed with overt PolyA.
  • Data collection included systematic patient interviews and medical record reviews using a detailed questionnaire covering demographic, clinical, immunological, and familial characteristics.
  • Hierarchical cluster analysis was employed to identify similarities and patterns among co-occurring autoimmune diseases.

Main Results:

  • PolyA occurred simultaneously in 44% of patients, with multiple autoimmune syndrome observed in 19.8%.
  • The most frequent index diseases were systemic lupus erythematosus (SLE, 42.8%), juvenile idiopathic arthritis (JIA, 12.7%), Hashimoto's thyroiditis (HT, 7.66%), immune thrombocytopenic purpura (ITP, 6.39%), antiphospholipid syndrome (APS, 4.79%), and vitiligo (VIT, 4.79%).
  • Familial autoimmunity was a significant factor, showing high aggregation (λr = 3.5). Three main disease clusters were identified, with SLE/APS, HT/JIA, and localized scleroderma/VIT showing specific similarities.

Conclusions:

  • The study highlights significant familial aggregation and distinct clustering patterns among autoimmune diseases in pediatric PolyA.
  • Systematic assessment for PolyA in pediatric patients with existing autoimmune diseases is recommended to facilitate early diagnosis and intervention.