Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review

BackgroundInterleukin (IL)-1 inhibitors represent the main treatment in patients with colchicine-resistant/intolerant familial Mediterranean fever (crFMF), mevalonate kinase deficiency (MKD), and tumor necrosis factor receptor-associated periodic syndrome (TRAPS). However, the reasons for the use of...

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Autores principales:	Caroline Vinit, Sophie Georgin-Lavialle, Aikaterini Theodoropoulou, Catherine Barbier, Alexandre Belot, Manel Mejbri, Pascal Pillet, Jana Pachlopnik, Sylvaine Poignant, Charlotte Rebelle, Andreas Woerner, Isabelle Koné-Paut, Véronique Hentgen
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	autoinflammatory diseases indications treatment hereditary recurrent fevers interleukin-1 blockers anakinra Immunologic diseases. Allergy RC581-607
Acceso en línea:	https://doaj.org/article/26ad2c1c806644ef91dc77d124b1103b
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spelling	oai:doaj.org-article:26ad2c1c806644ef91dc77d124b1103b2021-11-11T08:41:15ZReal-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review1664-322410.3389/fimmu.2021.744780https://doaj.org/article/26ad2c1c806644ef91dc77d124b1103b2021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fimmu.2021.744780/fullhttps://doaj.org/toc/1664-3224BackgroundInterleukin (IL)-1 inhibitors represent the main treatment in patients with colchicine-resistant/intolerant familial Mediterranean fever (crFMF), mevalonate kinase deficiency (MKD), and tumor necrosis factor receptor-associated periodic syndrome (TRAPS). However, the reasons for the use of IL-1 inhibitors in these diseases are still not completely clarified.ObjectiveIdentify real-life situations that led to initiating anakinra or canakinumab treatment in hereditary recurrent fevers (HRFs), combining data from an international registry and an up-to-date literature review.Patients and MethodsData were extracted from the JIRcohort, in which clinical information (demographic data, treatment, disease activity, and quality of life) on patients with FMF, MKD, and TRAPS was retrospectively collected. A literature search was conducted using Medline, EMBASE, and Cochrane databases.ResultsComplete data of 93 patients with HRF (53.8% FMF, 31.2% MKD, and 15.1% TRAPS) were analyzed. Data from both the registry and the literature review confirmed that the main reasons for use of IL-1 blockers were the following: failure of previous treatment (n = 57, 61.3% and n = 964, 75.3%, respectively), persistence of disease activity with frequent attacks (n = 44, 47.3% and n = 1,023, 79.9%) and/or uncontrolled inflammatory syndrome (n = 46, 49.5% and n = 398, 31.1%), severe disease complication or associated comorbidities (n = 38, 40.9% and n = 390, 30.4%), and worsening of patients’ quality of life (n = 36, 38.7% and n = 100, 7,8%). No reasons were specified for 12 (16.4%) JIRcohort patients and 154 (12%) patients in the literature.ConclusionIn the absence of standardized indications for IL-1 inhibitors in crFMF, MKD, and TRAPS, these results could serve as a basis for developing a treat-to-target strategy that would help clinicians codify the therapeutic escalation with IL-1 inhibitors.Caroline VinitCaroline VinitSophie Georgin-LavialleSophie Georgin-LavialleAikaterini TheodoropoulouAikaterini TheodoropoulouCatherine BarbierAlexandre BelotAlexandre BelotManel MejbriManel MejbriPascal PilletJana PachlopnikSylvaine PoignantCharlotte RebelleAndreas WoernerIsabelle Koné-PautIsabelle Koné-PautVéronique HentgenVéronique HentgenFrontiers Media S.A.articleautoinflammatory diseasesindicationstreatmenthereditary recurrent feversinterleukin-1 blockersanakinraImmunologic diseases. AllergyRC581-607ENFrontiers in Immunology, Vol 12 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	autoinflammatory diseases indications treatment hereditary recurrent fevers interleukin-1 blockers anakinra Immunologic diseases. Allergy RC581-607
spellingShingle	autoinflammatory diseases indications treatment hereditary recurrent fevers interleukin-1 blockers anakinra Immunologic diseases. Allergy RC581-607 Caroline Vinit Caroline Vinit Sophie Georgin-Lavialle Sophie Georgin-Lavialle Aikaterini Theodoropoulou Aikaterini Theodoropoulou Catherine Barbier Alexandre Belot Alexandre Belot Manel Mejbri Manel Mejbri Pascal Pillet Jana Pachlopnik Sylvaine Poignant Charlotte Rebelle Andreas Woerner Isabelle Koné-Paut Isabelle Koné-Paut Véronique Hentgen Véronique Hentgen Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review
description	BackgroundInterleukin (IL)-1 inhibitors represent the main treatment in patients with colchicine-resistant/intolerant familial Mediterranean fever (crFMF), mevalonate kinase deficiency (MKD), and tumor necrosis factor receptor-associated periodic syndrome (TRAPS). However, the reasons for the use of IL-1 inhibitors in these diseases are still not completely clarified.ObjectiveIdentify real-life situations that led to initiating anakinra or canakinumab treatment in hereditary recurrent fevers (HRFs), combining data from an international registry and an up-to-date literature review.Patients and MethodsData were extracted from the JIRcohort, in which clinical information (demographic data, treatment, disease activity, and quality of life) on patients with FMF, MKD, and TRAPS was retrospectively collected. A literature search was conducted using Medline, EMBASE, and Cochrane databases.ResultsComplete data of 93 patients with HRF (53.8% FMF, 31.2% MKD, and 15.1% TRAPS) were analyzed. Data from both the registry and the literature review confirmed that the main reasons for use of IL-1 blockers were the following: failure of previous treatment (n = 57, 61.3% and n = 964, 75.3%, respectively), persistence of disease activity with frequent attacks (n = 44, 47.3% and n = 1,023, 79.9%) and/or uncontrolled inflammatory syndrome (n = 46, 49.5% and n = 398, 31.1%), severe disease complication or associated comorbidities (n = 38, 40.9% and n = 390, 30.4%), and worsening of patients’ quality of life (n = 36, 38.7% and n = 100, 7,8%). No reasons were specified for 12 (16.4%) JIRcohort patients and 154 (12%) patients in the literature.ConclusionIn the absence of standardized indications for IL-1 inhibitors in crFMF, MKD, and TRAPS, these results could serve as a basis for developing a treat-to-target strategy that would help clinicians codify the therapeutic escalation with IL-1 inhibitors.
format	article
author	Caroline Vinit Caroline Vinit Sophie Georgin-Lavialle Sophie Georgin-Lavialle Aikaterini Theodoropoulou Aikaterini Theodoropoulou Catherine Barbier Alexandre Belot Alexandre Belot Manel Mejbri Manel Mejbri Pascal Pillet Jana Pachlopnik Sylvaine Poignant Charlotte Rebelle Andreas Woerner Isabelle Koné-Paut Isabelle Koné-Paut Véronique Hentgen Véronique Hentgen
author_facet	Caroline Vinit Caroline Vinit Sophie Georgin-Lavialle Sophie Georgin-Lavialle Aikaterini Theodoropoulou Aikaterini Theodoropoulou Catherine Barbier Alexandre Belot Alexandre Belot Manel Mejbri Manel Mejbri Pascal Pillet Jana Pachlopnik Sylvaine Poignant Charlotte Rebelle Andreas Woerner Isabelle Koné-Paut Isabelle Koné-Paut Véronique Hentgen Véronique Hentgen
author_sort	Caroline Vinit
title	Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review
title_short	Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review
title_full	Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review
title_fullStr	Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review
title_full_unstemmed	Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review
title_sort	real-life indications of interleukin-1 blocking agents in hereditary recurrent fevers: data from the jircohort and a literature review
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/26ad2c1c806644ef91dc77d124b1103b
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Real-Life Indications of Interleukin-1 Blocking Agents in Hereditary Recurrent Fevers: Data From the JIRcohort and a Literature Review

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