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Pediatric Recurrent Pericarditis: Appropriateness of the Standard of Care and Response to IL-1 Blockade

Roberta Caorsi1, Antonella Insalaco2, Francesca Bovis3

  • 1Center of Autoinflammatory Diseases and Immunodeficiencies, Department of Pediatrics and Rheumatology, IRCCS Istituto G. Gaslini, Genova, Italy.

The Journal of Pediatrics
|December 5, 2022
PubMed

Insights

Anti-interleukin (IL)-1 treatment for pediatric recurrent pericarditis often involves inadequate dosing. While anakinra is effective, achieving drug-free remission remains challenging, with shorter treatment durations linked to better outcomes.

Area of Science:

  • Pediatric Rheumatology
  • Immunology
  • Cardiology

Background:

  • Recurrent pericarditis in children often requires advanced therapies.
  • Interleukin-1 (IL-1) blockade has emerged as a treatment option.
  • Optimal dosing and long-term outcomes of IL-1 inhibitors in pediatric recurrent pericarditis are not well-established.

Purpose of the Study:

  • To analyze the efficacy of anti-IL-1 treatment in pediatric recurrent pericarditis.
  • To determine the rate of drug-free remission and associated factors.
  • To evaluate the agents and dosing strategies used in first-line treatment.

Main Methods:

  • Retrospective analysis of pediatric patients with recurrent pericarditis treated with IL-1 blockers.
  • Evaluation of treatment efficacy using annualized relapse rate (ARR).
  • Bivariate logistic regression to identify variables associated with drug-free remission.

Main Results:

  • Most patients received inadequate first-line anti-IL-1 therapy.
  • Anakinra significantly reduced ARR (3.05 to 0.28, P < .0001), but relapse increased upon withdrawal (to 0.83, P < .0001).
  • Only 9 of 58 patients achieved drug-free remission; shorter anakinra duration was associated with remission.

Conclusions:

  • First-line anti-IL-1 treatment for pediatric recurrent pericarditis is frequently underdosed.
  • Anakinra demonstrates effectiveness, but achieving drug-free remission is uncommon.
  • Differences in response between anakinra and canakinumab suggest a potential role for IL-1α in disease pathogenesis.
Abstract

Keywords:
anti-inflammatory agents nonsteroidalcolchicineinterleukin-1pericarditissteroidstherapy

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