Abstract
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder that is characterised by high spiking fever, arthritis or arthralgia, and evanescent rash. Many other systemic manifestations may occur. Pathogenesis of AOSD remains partially unknown but a major role has been recently attributed to pro-inflammatory Th1 cytokines, including tumour necrosis factor-α (TNF-α), interleukin (IL)-1, IL-6 and IL-18. Despite limited evidence, mainly based on observational studies and the extrapolation to AOSD of the results of a few controlled studies that have been conducted in children with systemic juvenile idiopathic arthritis, biological agents represent a major therapeutic advances for patients with AOSD refractory to conventional treatment or presenting life-threatening manifestations. Both IL-1 and IL-6 blockade may be more effective than TNF-α blockers. Although debatable, therapeutic strategies are proposed.
Copyright © 2012 Elsevier Ltd. All rights reserved.
MeSH terms
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Adalimumab
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Antibodies, Monoclonal / therapeutic use
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Antibodies, Monoclonal, Humanized / therapeutic use
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Antirheumatic Agents / therapeutic use*
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Cytokines / therapeutic use
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Etanercept
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Exanthema / complications
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Exanthema / pathology
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Humans
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Immunoglobulin G / therapeutic use
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Infliximab
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Interleukin 1 Receptor Antagonist Protein / therapeutic use
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Interleukin-1 / antagonists & inhibitors
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Interleukin-18 / antagonists & inhibitors
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Interleukin-6 / antagonists & inhibitors
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Methotrexate / therapeutic use
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Rare Diseases / drug therapy*
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Rare Diseases / etiology
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Rare Diseases / pathology
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Receptors, Tumor Necrosis Factor / therapeutic use
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Still's Disease, Adult-Onset / drug therapy*
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Still's Disease, Adult-Onset / etiology
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Still's Disease, Adult-Onset / pathology
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Tumor Necrosis Factor-alpha / antagonists & inhibitors
Substances
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Antibodies, Monoclonal
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Antibodies, Monoclonal, Humanized
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Antirheumatic Agents
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Cytokines
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Immunoglobulin G
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Interleukin 1 Receptor Antagonist Protein
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Interleukin-1
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Interleukin-18
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Interleukin-6
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Receptors, Tumor Necrosis Factor
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Tumor Necrosis Factor-alpha
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Infliximab
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Adalimumab
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Etanercept
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Methotrexate