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Title:
Pathogenesis of adult-onset Still鈥檚 disease: new insights from the juvenile counterpart | Immunologic Research
Description:
Adult-onset Still鈥檚 disease (AOSD) is a rare inflammatory disease characterized by the classical triad of daily fever, arthritis, and typical salmon-colored rash. Recent accumulation of knowledge, mostly arising from hereditary autoinflammatory diseases and from the systemic-onset juvenile idiopathic arthritis (sJIA), has given raise to new hypotheses on the pathophysiology of AOSD. In this review, we first discuss on the continuum between AOSD and sJIA. Then, we summarize current hypotheses on the underlying pathogenesis: (1) an infectious hypothesis; (2) an autoinflammatory hypothesis; (3) a lymphohistiocytic hypothesis; and (4) a hyperferritinemic hypothesis. Finally, we present the recent data suggesting that patients with AOSD fall into two distinct subgroups with different courses, one with prominent systemic features and one with chronic arthritis.
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Keywords {馃攳}
pubmed, google, scholar, article, cas, disease, arthritis, stills, juvenile, idiopathic, adultonset, patients, central, rheumatol, systemic, rheum, adult, onset, syndrome, clin, activation, autoinflammatory, dis, immunol, macrophage, jamilloux, chen, pathogenesis, clinical, gene, rev, ann, lee, kim, interleukin, yvan, systemiconset, treatment, cell, res, research, gerfaudvalentin, association, role, cytokine, profiles, henry, cells, natural, killer,
Topics {鉁掞笍}
hemophagocytic lymphohistiocytosis targeting interleukin-1尾 perforin-mediated target-cell death month download article/chapter mathieu gerfaud-valentin reactive haemophagocytic syndrome secondary hemophagocytic syndrome author information authors alexandre belot iron-independent protein kinase macrophage-colony stimulating factor tnf-alpha-producing macrophages mhc-encoded lmp7 gene typical salmon-colored rash liver biopsy illustrating h么pital femme-m猫re-enfant hereditary autoinflammatory diseases monogenic autoinflammatory diseases juvenile idiopathic arthritis ifn-gamma-producing lymphocytes circulating cd4聽+聽cd25high regulatory author correspondence anti-interleukin-1 treatment distinguishes il-18/il-18bp gerfaud-valentin serum cytokine profiles full article pdf autoinflammatory diseases plasma il-6 levels line disease-modifying therapy il-1 gene family phd research project proinflammatory cytokine profiles distinct cytokine profiles related subjects check access instant access nlrp3 inflammasome gene expression profiling myeloid-related proteins 8 privacy choices/manage cookies cell death dis autoinflammatory syndromes goldbach-mansky park d pascal s猫ve yvan jamilloux acknowledges nk cell function autoinflammatory disease reloaded ogilvie em
Questions {鉂搣
- Ferritin in adult-onset still鈥檚 disease: just a useful innocent bystander?
- How should we approach classification of autoinflammatory diseases?
- Is Still鈥檚 disease an autoinflammatory syndrome?
- Still鈥檚 disease and the mitochondrion: the other face of an old friend?
- Targeting interleukin-1尾 in CAPS (cryopyrin-associated periodic) syndromes: what did we learn?
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headline:Pathogenesis of adult-onset Still鈥檚 disease: new insights from the juvenile counterpart
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Adult-onset Still鈥檚 disease (AOSD) is a rare inflammatory disease characterized by the classical triad of daily fever, arthritis, and typical salmon-colored rash. Recent accumulation of knowledge, mostly arising from hereditary autoinflammatory diseases and from the systemic-onset juvenile idiopathic arthritis (sJIA), has given raise to new hypotheses on the pathophysiology of AOSD. In this review, we first discuss on the continuum between AOSD and sJIA. Then, we summarize current hypotheses on the underlying pathogenesis: (1) an infectious hypothesis; (2) an autoinflammatory hypothesis; (3) a lymphohistiocytic hypothesis; and (4) a hyperferritinemic hypothesis. Finally, we present the recent data suggesting that patients with AOSD fall into two distinct subgroups with different courses, one with prominent systemic features and one with chronic arthritis.
datePublished:2014-11-12T00:00:00Z
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Adult-onset Still鈥檚 disease
Systemic-onset juvenile idiopathic arthritis
Autoinflammatory diseases
Reactive hemophagocytic lymphohistiocytosis
IL-1尾
IL-18
Ferritin
Immunology
Allergology
Medicine/Public Health
general
Internal Medicine
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headline:Pathogenesis of adult-onset Still鈥檚 disease: new insights from the juvenile counterpart
description:
Adult-onset Still鈥檚 disease (AOSD) is a rare inflammatory disease characterized by the classical triad of daily fever, arthritis, and typical salmon-colored rash. Recent accumulation of knowledge, mostly arising from hereditary autoinflammatory diseases and from the systemic-onset juvenile idiopathic arthritis (sJIA), has given raise to new hypotheses on the pathophysiology of AOSD. In this review, we first discuss on the continuum between AOSD and sJIA. Then, we summarize current hypotheses on the underlying pathogenesis: (1) an infectious hypothesis; (2) an autoinflammatory hypothesis; (3) a lymphohistiocytic hypothesis; and (4) a hyperferritinemic hypothesis. Finally, we present the recent data suggesting that patients with AOSD fall into two distinct subgroups with different courses, one with prominent systemic features and one with chronic arthritis.
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Adult-onset Still鈥檚 disease
Systemic-onset juvenile idiopathic arthritis
Autoinflammatory diseases
Reactive hemophagocytic lymphohistiocytosis
IL-1尾
IL-18
Ferritin
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Allergology
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