IL-1 Inhibitors in the Treatment of Monogenic Periodic Fever Syndromes: From the Past to the Future Perspectives
Autoinflammatory diseases (AIDs) represent a rare and heterogeneous group of disorders characterized by recurrent episodes of inflammation and a broad range of clinical manifestations. The most common symptoms involve recurrent fevers, musculoskeletal symptoms, and serositis; however, AIDs can also...
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doaj-8f05417ca6ff486da3e765abf15f3e302021-02-02T07:40:20ZengFrontiers Media S.A.Frontiers in Immunology1664-32242021-02-011110.3389/fimmu.2020.619257619257IL-1 Inhibitors in the Treatment of Monogenic Periodic Fever Syndromes: From the Past to the Future PerspectivesHana Malcova0Zuzana Strizova1Tomas Milota2Tomas Milota3Ilja Striz4Anna Sediva5Dita Cebecauerova6Rudolf Horvath7Rudolf Horvath8Department of Paediatric and Adult Rheumatology, University Hospital Motol, Prague, CzechiaDepartment of Immunology, Second Faculty of Medicine Charles University and University Hospital Motol, Prague, CzechiaDepartment of Paediatric and Adult Rheumatology, University Hospital Motol, Prague, CzechiaDepartment of Immunology, Second Faculty of Medicine Charles University and University Hospital Motol, Prague, CzechiaDepartment of Clinical Immunology and Allergology, Institute for Clinical and Experimental Medicine, Prague, CzechiaDepartment of Immunology, Second Faculty of Medicine Charles University and University Hospital Motol, Prague, CzechiaDepartment of Paediatric and Adult Rheumatology, University Hospital Motol, Prague, CzechiaDepartment of Paediatric and Adult Rheumatology, University Hospital Motol, Prague, CzechiaDepartment of Immunology, Second Faculty of Medicine Charles University and University Hospital Motol, Prague, CzechiaAutoinflammatory diseases (AIDs) represent a rare and heterogeneous group of disorders characterized by recurrent episodes of inflammation and a broad range of clinical manifestations. The most common symptoms involve recurrent fevers, musculoskeletal symptoms, and serositis; however, AIDs can also lead to life-threatening complications, such as macrophage activation syndrome (MAS) and systemic AA amyloidosis. Typical monogenic periodic fever syndromes include cryopyrin-associated periodic fever syndrome (CAPS), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency/hyper IgD syndrome (MKD/HIDS), and familial Mediterranean fever (FMF). However, a number of other clinical entities, such as systemic juvenile idiopathic arthritis (sJIA), adult-onset Still’s disease (AOSD), Kawasaki disease (KD) and idiopathic recurrent pericarditis (IRP), display similar phenotypical and immunological features to AIDs. All these diseases are pathophysiologicaly characterized by dysregulation of the innate immune system and the central pathogenic role is attributed to the IL-1 cytokine family (IL-1α, IL-1β, IL-1Ra, IL-18, IL-36Ra, IL-36α, IL-37, IL-36β, IL-36g, IL-38, and IL-33). Therefore, reasonable therapeutic approaches aim to inhibit these cytokines and their pathways. To date, several anti-IL-1 therapies have evolved. Each drug differs in structure, mechanism of action, efficacy for the treatment of selected diseases, and side effects. Most of the available data regarding the efficacy and safety of IL-1 inhibitors are related to anakinra, canakinumab, and rilonacept. Other promising therapeutics, such as gevokizumab, tadekinig alfa, and tranilast are currently undergoing clinical trials. In this review, we provide sophisticated and up-to-date insight into the therapeutic uses of different IL-1 inhibitors in monogenic periodic fever syndromes.https://www.frontiersin.org/articles/10.3389/fimmu.2020.619257/fullIL-1anakinracanakinumabrilonaceptCAPSTRAPS |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Hana Malcova Zuzana Strizova Tomas Milota Tomas Milota Ilja Striz Anna Sediva Dita Cebecauerova Rudolf Horvath Rudolf Horvath |
spellingShingle |
Hana Malcova Zuzana Strizova Tomas Milota Tomas Milota Ilja Striz Anna Sediva Dita Cebecauerova Rudolf Horvath Rudolf Horvath IL-1 Inhibitors in the Treatment of Monogenic Periodic Fever Syndromes: From the Past to the Future Perspectives Frontiers in Immunology IL-1 anakinra canakinumab rilonacept CAPS TRAPS |
author_facet |
Hana Malcova Zuzana Strizova Tomas Milota Tomas Milota Ilja Striz Anna Sediva Dita Cebecauerova Rudolf Horvath Rudolf Horvath |
author_sort |
Hana Malcova |
title |
IL-1 Inhibitors in the Treatment of Monogenic Periodic Fever Syndromes: From the Past to the Future Perspectives |
title_short |
IL-1 Inhibitors in the Treatment of Monogenic Periodic Fever Syndromes: From the Past to the Future Perspectives |
title_full |
IL-1 Inhibitors in the Treatment of Monogenic Periodic Fever Syndromes: From the Past to the Future Perspectives |
title_fullStr |
IL-1 Inhibitors in the Treatment of Monogenic Periodic Fever Syndromes: From the Past to the Future Perspectives |
title_full_unstemmed |
IL-1 Inhibitors in the Treatment of Monogenic Periodic Fever Syndromes: From the Past to the Future Perspectives |
title_sort |
il-1 inhibitors in the treatment of monogenic periodic fever syndromes: from the past to the future perspectives |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Immunology |
issn |
1664-3224 |
publishDate |
2021-02-01 |
description |
Autoinflammatory diseases (AIDs) represent a rare and heterogeneous group of disorders characterized by recurrent episodes of inflammation and a broad range of clinical manifestations. The most common symptoms involve recurrent fevers, musculoskeletal symptoms, and serositis; however, AIDs can also lead to life-threatening complications, such as macrophage activation syndrome (MAS) and systemic AA amyloidosis. Typical monogenic periodic fever syndromes include cryopyrin-associated periodic fever syndrome (CAPS), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency/hyper IgD syndrome (MKD/HIDS), and familial Mediterranean fever (FMF). However, a number of other clinical entities, such as systemic juvenile idiopathic arthritis (sJIA), adult-onset Still’s disease (AOSD), Kawasaki disease (KD) and idiopathic recurrent pericarditis (IRP), display similar phenotypical and immunological features to AIDs. All these diseases are pathophysiologicaly characterized by dysregulation of the innate immune system and the central pathogenic role is attributed to the IL-1 cytokine family (IL-1α, IL-1β, IL-1Ra, IL-18, IL-36Ra, IL-36α, IL-37, IL-36β, IL-36g, IL-38, and IL-33). Therefore, reasonable therapeutic approaches aim to inhibit these cytokines and their pathways. To date, several anti-IL-1 therapies have evolved. Each drug differs in structure, mechanism of action, efficacy for the treatment of selected diseases, and side effects. Most of the available data regarding the efficacy and safety of IL-1 inhibitors are related to anakinra, canakinumab, and rilonacept. Other promising therapeutics, such as gevokizumab, tadekinig alfa, and tranilast are currently undergoing clinical trials. In this review, we provide sophisticated and up-to-date insight into the therapeutic uses of different IL-1 inhibitors in monogenic periodic fever syndromes. |
topic |
IL-1 anakinra canakinumab rilonacept CAPS TRAPS |
url |
https://www.frontiersin.org/articles/10.3389/fimmu.2020.619257/full |
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