Systemic juvenile idiopathic arthritis

New insights into pathogenesis and cytokine directed therapies

Research output: Contribution to journalReview article

Abstract

Systemic juvenile idiopathic arthritis (sJIA) is considered as a polygenic autoinflammatory disease. The prominent systemic clinical features, the marked elevation of inflammatory markers, and the absence of autoantibodies make this disease very different from the other juvenile idiopathic arthritis (JIA) forms. Innate immune mechanisms appear to play a central role: the overproduction of inflammatory cytokines of innate immunity is a typical feature of sJIA. Increased understanding of the role of these cytokines has been translated into therapeutic approaches. Indeed, remarkable results have been observed with interleukin-1 (IL-1) and interleukin-6 (IL-6) inhibitors both in clinical trials and in real life. Other inhibitors of these cytokines will be available in the near future, thereby increasing the therapeutic armamentarium. A better understanding of the pathophysiology of sJIA is still needed to identify IL-1 or IL-6 responders, define a potential window of opportunity for early cytokine blockade, and identify a targeted treatment for macrophage activation syndrome. Additional therapeutic targets are needed for a small proportion of patients who do not respond to either IL-1 or IL-6 inhibition.

Original languageEnglish
Pages (from-to)505-516
Number of pages12
JournalBest Practice and Research in Clinical Rheumatology
Volume31
Issue number4
DOIs
Publication statusPublished - 2018

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Juvenile Arthritis
Interleukin-1
Cytokines
Interleukin-6
Macrophage Activation Syndrome
Therapeutics
Innate Immunity
Autoantibodies
Clinical Trials

Cite this

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title = "Systemic juvenile idiopathic arthritis: New insights into pathogenesis and cytokine directed therapies",
abstract = "Systemic juvenile idiopathic arthritis (sJIA) is considered as a polygenic autoinflammatory disease. The prominent systemic clinical features, the marked elevation of inflammatory markers, and the absence of autoantibodies make this disease very different from the other juvenile idiopathic arthritis (JIA) forms. Innate immune mechanisms appear to play a central role: the overproduction of inflammatory cytokines of innate immunity is a typical feature of sJIA. Increased understanding of the role of these cytokines has been translated into therapeutic approaches. Indeed, remarkable results have been observed with interleukin-1 (IL-1) and interleukin-6 (IL-6) inhibitors both in clinical trials and in real life. Other inhibitors of these cytokines will be available in the near future, thereby increasing the therapeutic armamentarium. A better understanding of the pathophysiology of sJIA is still needed to identify IL-1 or IL-6 responders, define a potential window of opportunity for early cytokine blockade, and identify a targeted treatment for macrophage activation syndrome. Additional therapeutic targets are needed for a small proportion of patients who do not respond to either IL-1 or IL-6 inhibition.",
author = "Manuela Pardeo and Claudia Bracaglia and {De Benedetti}, Fabrizio",
note = "Copyright {\circledC} 2018 Elsevier Ltd. All rights reserved.",
year = "2018",
doi = "10.1016/j.berh.2018.02.002",
language = "English",
volume = "31",
pages = "505--516",
journal = "Best Practice and Research in Clinical Rheumatology",
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T1 - Systemic juvenile idiopathic arthritis

T2 - New insights into pathogenesis and cytokine directed therapies

AU - Pardeo, Manuela

AU - Bracaglia, Claudia

AU - De Benedetti, Fabrizio

N1 - Copyright © 2018 Elsevier Ltd. All rights reserved.

PY - 2018

Y1 - 2018

N2 - Systemic juvenile idiopathic arthritis (sJIA) is considered as a polygenic autoinflammatory disease. The prominent systemic clinical features, the marked elevation of inflammatory markers, and the absence of autoantibodies make this disease very different from the other juvenile idiopathic arthritis (JIA) forms. Innate immune mechanisms appear to play a central role: the overproduction of inflammatory cytokines of innate immunity is a typical feature of sJIA. Increased understanding of the role of these cytokines has been translated into therapeutic approaches. Indeed, remarkable results have been observed with interleukin-1 (IL-1) and interleukin-6 (IL-6) inhibitors both in clinical trials and in real life. Other inhibitors of these cytokines will be available in the near future, thereby increasing the therapeutic armamentarium. A better understanding of the pathophysiology of sJIA is still needed to identify IL-1 or IL-6 responders, define a potential window of opportunity for early cytokine blockade, and identify a targeted treatment for macrophage activation syndrome. Additional therapeutic targets are needed for a small proportion of patients who do not respond to either IL-1 or IL-6 inhibition.

AB - Systemic juvenile idiopathic arthritis (sJIA) is considered as a polygenic autoinflammatory disease. The prominent systemic clinical features, the marked elevation of inflammatory markers, and the absence of autoantibodies make this disease very different from the other juvenile idiopathic arthritis (JIA) forms. Innate immune mechanisms appear to play a central role: the overproduction of inflammatory cytokines of innate immunity is a typical feature of sJIA. Increased understanding of the role of these cytokines has been translated into therapeutic approaches. Indeed, remarkable results have been observed with interleukin-1 (IL-1) and interleukin-6 (IL-6) inhibitors both in clinical trials and in real life. Other inhibitors of these cytokines will be available in the near future, thereby increasing the therapeutic armamentarium. A better understanding of the pathophysiology of sJIA is still needed to identify IL-1 or IL-6 responders, define a potential window of opportunity for early cytokine blockade, and identify a targeted treatment for macrophage activation syndrome. Additional therapeutic targets are needed for a small proportion of patients who do not respond to either IL-1 or IL-6 inhibition.

U2 - 10.1016/j.berh.2018.02.002

DO - 10.1016/j.berh.2018.02.002

M3 - Review article

VL - 31

SP - 505

EP - 516

JO - Best Practice and Research in Clinical Rheumatology

JF - Best Practice and Research in Clinical Rheumatology

SN - 1521-6942

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ER -