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

• Published in: Frontiers in immunology Vol. 12; p. 744780
• Main Authors: Vinit, Caroline, Georgin-Lavialle, Sophie, Theodoropoulou, Aikaterini, Barbier, Catherine, Belot, Alexandre, Mejbri, Manel, Pillet, Pascal, Pachlopnik, Jana, Poignant, Sylvaine, Rebelle, Charlotte, Woerner, Andreas, Koné-Paut, Isabelle, Hentgen, Véronique
• Format: Journal Article
• Language: English
• Published: Switzerland Frontiers 11-11-2021; Frontiers Media S.A
• Subjects: Adolescent; Adult; Aged; anakinra; Antibodies, Monoclonal, Humanized - therapeutic use; Antirheumatic Agents - therapeutic use; autoinflammatory diseases; Child; Child, Preschool; Female; Hereditary Autoinflammatory Diseases - complications; Hereditary Autoinflammatory Diseases - drug therapy; hereditary recurrent fevers; Humans; Immunology; indications; Infant; Interleukin 1 Receptor Antagonist Protein - therapeutic use; Interleukin-1 - antagonists & inhibitors; interleukin-1 blockers; Life Sciences; Male; Middle Aged; Retrospective Studies; treatment; Young Adult; anakinra; treatment; indications; canakinumab; autoinflammatory diseases; interleukin-1 blockers; hereditary recurrent fevers
• ISSN: 1664-3224; 1664-3224
• DOI: 10.3389/fimmu.2021.744780

Interleukin (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.
Identify 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.
Data 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.
Complete 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.
In 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.