Long-Term Follow-Up and Optimization of Interleukin-1 Inhibitors in the Management of Monogenic Autoinflammatory Diseases: Real-Life Data from the JIR Cohort.

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License: CC BY 4.0
Serval ID
serval:BIB_DCE42A29732E
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Long-Term Follow-Up and Optimization of Interleukin-1 Inhibitors in the Management of Monogenic Autoinflammatory Diseases: Real-Life Data from the JIR Cohort.
Journal
Frontiers in pharmacology
Author(s)
Hentgen V., Koné-Paut I., Belot A., Galeotti C., Grateau G., Carbasse A., Pagnier A., Pillet P., Delord M., Hofer M., Georgin-Lavialle S.
ISSN
1663-9812 (Print)
ISSN-L
1663-9812
Publication state
Published
Issued date
2020
Peer-reviewed
Oui
Volume
11
Pages
568865
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
Objectives: The major role of interleukin (IL)-1 in the pathogenesis of hereditary recurrent fever syndromes favored the employment of targeted therapies modulating IL-1 signaling. However the best use of IL1 inhibitors in terms of dosage is difficult to define at present. 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 (Familial Mediterranean Fever (FMF), TNF receptor associated periodic syndrome (TRAPS), cryopyrin associated periodic fever (CAPS) and mevalonate kinase deficiency). The patients were retrieved retrospectively from the JIR cohort, an international platform gathering data of patients with pediatric inflammatory diseases. Results: Forty five patients of the JIR cohort with a hereditary recurrent fever syndrome 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. Especially patients with FMF and TRAPS seemed to need lower treatment regimens; 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 two 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 mevalonate kinase deficiency 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. Severity, activity but also the type of the underlying disease, belong to the parameters underpinning the treat-to-target strategy implemented in an everyday life practice.
Keywords
IL-1 inhibitor, Tumor Necrosis factor (TNF)-receptor-associated periodic syndrome, anakinra, canakinumab, cryopyrin-associated periodic syndrome, familial mediterranean fever disease, mevalonate kinase deficiency, posology
Pubmed
Web of science
Open Access
Yes
Create date
08/02/2021 17:01
Last modification date
23/01/2024 8:35
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