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Interleukin 10 and interleukin 10 receptor in paediatric inflammatory bowel disease: from bench to bedside lesson

Paulina Krawiec1, Agnieszka Pawłowska-Kamieniak2, Elżbieta Pac-Kożuchowska2

  • 1Department of Paediatrics and Gastroenterology, Medical University of Lublin, Racławickie 1, 20-059, Lublin, Poland. paulinakrawiec@umlub.pl.

Journal of Inflammation (London, England)
|March 11, 2021
PubMed

Insights

Genetic defects in Interleukin-10 (IL-10) signaling cause severe early-onset inflammatory bowel disease (IBD) in children. Early recognition and allogeneic hematopoietic stem cell transplantation offer curative treatment for these monogenic IBD cases.

Area of Science:

  • Pediatric Gastroenterology
  • Immunology
  • Genetics

Background:

  • Inflammatory bowel disease (IBD) presents differently in children compared to adults, leading to the distinct field of pediatric IBD.
  • In young children (under 6 years), IBD is often caused by single gene mutations (monogenic IBD), while older children typically have polygenic IBD.
  • Over 60 monogenic defects impacting immune mechanisms have been identified in IBD pathogenesis.

Purpose of the Study:

  • To highlight the role of Interleukin-10 (IL-10) signaling defects in early-onset pediatric IBD.
  • To emphasize the clinical presentation and curative treatment options for IL-10 pathway defects.

Main Methods:

  • Review of known monogenic defects in IBD pathogenesis.
  • Focus on the function of IL-10 as a key anti-inflammatory cytokine.
  • Description of clinical manifestations in patients with IL-10 pathway defects.

Main Results:

  • Defects in the IL-10 pathway lead to severe, life-threatening colitis with perianal lesions presenting in early infancy.
  • IL-10 modulates both innate and adaptive immunity, affecting pro-inflammatory molecule expression and immune cell function.
  • Allogeneic hematopoietic stem cell transplantation is a curative therapy for children with IL-10 signaling defects.

Conclusions:

  • Clinical awareness of IL-10 signaling defects is crucial for early diagnosis.
  • Prompt management, including consideration of stem cell transplantation, can significantly improve outcomes for affected children.
Abstract

Keywords:
Crohn’s diseaseGeneticsImmunityUlcerative colitis

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