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Brief Research Report ARTICLE

Front. Pharmacol. | doi: 10.3389/fphar.2020.568865

IL-1 inhibitor use in recurrent fever syndromes: a study on dosing from the JIR cohort Provisionally accepted The final, formatted version of the article will be published soon. Notify me

 Véronique Hentgen1, 2*,  Isabelle Kone-paut2, 3,  Alexandre Belot4, 5, 6,  Caroline Galeotti2, 3, Gilles Grateau2, 7, Aurelia Carbasse8, Anne Pagnier9, Pascal Pillet10,  Michaël Hofer11 and  Sophie A. Georgin-Lavialle2, 7, 12
  • 1departement of pediatrics, Centre Hospitalier de Versailles, France
  • 2Centre de référence des Maladies Auto-Inflammatoires et de l’Amylose Inflammatoire, Hôpitaux Universitaires Paris-Sud (APHP), France
  • 3Bicêtre Hospital, France
  • 4Département de Rhumatologie, Hospices Civils de Lyon, France
  • 5Centre de Référence des Rhumatismes Inflammatoires et Maladies Auto-immunes Systémiques Rares de l’Enfant (RAISE), France
  • 6UMR5308 Centre International de Recherche en Infectiologie (CIRI), France
  • 7Department of internal medicine, Hôpital Tenon AP-HP, France
  • 8department of pediatrics, Hôpital Arnaud De Villeneuve, France
  • 9Department of pediatrics, Centre Hospitalier Universitaire de Grenoble, France
  • 10Department of pediatrics, Hôpital des Enfants, Centre Hospitalier Universitaire de Bordeaux, France
  • 11Unité Romande d’Immuno-Rhumatologie pédiatrique, Centre Hospitalier Universitaire Vaudois (CHUV), Switzerland
  • 12Université Paris-Sorbonne, France

Methods: In order to better understand the use of IL1 inhibitors in a real-life setting, our study assessed the dosage regimens of French patients with one of the four main hereditary recurrent fever syndromes (FMF, TRAPS, CAPS and MKD). The patients were retrieved retrospectively from the JIRcohort, an international platform gathering data of patients with pediatric inflammatory diseases.
Results: 45 patients had received at least once an IL1 inhibitor (anakinra or canakinumab). Of these, 43% received a lower dosage than the one suggested in the product recommendations, regardless of the type of the IL1 inhibitor. In our cohort none of the FMF or TRAPS patients received an intensified dose of IL-inhibitor. On-demand treatment with a short half-life IL-1 inhibitor has also been used successfully for some patients with one of these 2 conditions The standard dose was given to 42% of the patients; whereas an intensified dose of IL-1 inhibitors was given to 15% of the patients (44% of CAPS patients and 17% of MKD patients). In our cohort each individual patient’s need for treatment seemed highly variable, ranging from on demand treatment regimens to intensified dosage maintenance therapies depending on the activity and the severity of the underlying disease.
Conclusion: IL-1 inhibitors are a good treatment option for patients with a hereditary recurrent fever syndrome, but the individual need of the dosage of IL-1 inhibitors to control the disease effectively seems highly variable and depending on severity, activity but also the type of the underlying disease.

Keywords: anakinra, Canakinumab, CAPS, TRAPS (TNF-Receptor-Associated Periodic Syndrome), MKD, Posology, FMF disease

Received: 02 Jun 2020; Accepted: 12 Oct 2020.

Copyright: © 2020 Hentgen, Kone-paut, Belot, Galeotti, Grateau, Carbasse, Pagnier, Pillet, Hofer and Georgin-Lavialle. 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) and the copyright owner(s) 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: Dr. Véronique Hentgen, Centre Hospitalier de Versailles, departement of pediatrics, Le Chesnay, France,