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REVIEW article

Front. Pediatr.
Sec. Pediatric Immunology
Volume 12 - 2024 | doi: 10.3389/fped.2024.1448176
This article is part of the Research Topic Reviews in Pediatric Primary Immunodeficiencies View all 10 articles

The Riddle of Recurrent Fever: A clinical approach to Pediatric Autoinflammatory Diseases

Provisionally accepted
Bram Meertens Bram Meertens 1,2Levi Hoste Levi Hoste 1,2Simon J. Tavernier Simon J. Tavernier 2,3,4,5Filomeen Haerynck Filomeen Haerynck 1,2*
  • 1 Department of Pediatrics, Ghent University Hospital, Ghent, East Flanders, Belgium
  • 2 Jeffrey Modell Diagnostic Center, Rainbow Babies & Children's Hospital, Ghent, East Flanders, Belgium
  • 3 Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium
  • 4 Department of Biomedical Molecular Biology, Faculty of Sciences, Ghent University, Ghent, East Flanders, Belgium
  • 5 Center for Inflammation Research, Laboratory of Molecular Signal Transduction in Inflammation, Ghent, Belgium

The final, formatted version of the article will be published soon.

    Autoinflammatory diseases (AIDs) are a group of immunodysregulatory disorders resulting in the increased release or signaling of pro-inflammatory cytokines. Patients with AIDs present systemic inflammation in sterile conditions, which are mainly caused by defects in the innate immune system. Fever is one of the typical symptoms of this derailed immune signaling. In addition, autoinflammatory diseases manifest with varying other symptoms during flare-ups and interphasic periods. The diagnosis of these rare diseases poses numerous challenges. This paper provides an overview of AIDs that arise in childhood and in which fever commonly presents as a symptom. It outlines clinical signs, pathophysiology, diagnosis, and management for each syndrome. Additionally, we discuss a comprehensive diagnostic approach for children where an AID is suspected.

    Keywords: inborn errors of immunity, monogenic, Recurrent fever, Periodic fever, Autoinflammatory diseases, Autoinflammation, Inflammasome, pediatric

    Received: 12 Jun 2024; Accepted: 18 Sep 2024.

    Copyright: © 2024 Meertens, Hoste, Tavernier and Haerynck. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Filomeen Haerynck, Jeffrey Modell Diagnostic Center, Rainbow Babies & Children's Hospital, Ghent, East Flanders, Belgium

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.