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- W2163965009 endingPage "874" @default.
- W2163965009 startingPage "823" @default.
- W2163965009 abstract "Patients with autoinflammatory diseases present with noninfectious fever flares and systemic and/or disease-specific organ inflammation. Their excessive proinflammatory cytokine and chemokine responses can be life threatening and lead to organ damage over time. Studying such patients has revealed genetic defects that have helped unravel key innate immune pathways, including excessive IL-1 signaling, constitutive NF-κB activation, and, more recently, chronic type I IFN signaling. Discoveries of monogenic defects that lead to activation of proinflammatory cytokines have inspired the use of anticytokine-directed treatment approaches that have been life changing for many patients and have led to the approval of IL-1-blocking agents for a number of autoinflammatory conditions. In this review, we describe the genetically characterized autoinflammatory diseases, we summarize our understanding of the molecular pathways that drive clinical phenotypes and that continue to inspire the search for novel treatment targets, and we provide a conceptual framework for classification." @default.
- W2163965009 created "2016-06-24" @default.
- W2163965009 creator A5053367602 @default.
- W2163965009 creator A5054255771 @default.
- W2163965009 creator A5058152911 @default.
- W2163965009 creator A5077699690 @default.
- W2163965009 date "2015-03-21" @default.
- W2163965009 modified "2023-10-02" @default.
- W2163965009 title "Molecular Mechanisms in Genetically Defined Autoinflammatory Diseases: Disorders of Amplified Danger Signaling" @default.
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