Journal
ANNUAL REVIEW OF IMMUNOLOGY VOL 33
Volume 33, Issue -, Pages 823-874Publisher
ANNUAL REVIEWS
DOI: 10.1146/annurev-immunol-032414-112227
Keywords
autoinflammatory diseases; IL-I-mediated autoinflammatory diseases; IFN-mediated autoinflammatorv diseases; cryopyrin-associated periodic syndromes; proteasome-associated autoinflammatory syndromes; inflammasomes in human disease; hereditary fever syndromes; macrophage activation syndrome
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Funding
- Intramural NIH HHS [Z99 AR999999, ZIA AR041138-12] Funding Source: Medline
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Patients with autoinflammatory diseases present with noninfectious fever Hares 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-kappa B activation, and, more recently, chronic type I IFN signaling. Discoveries of monogenic defects that lead to activation of proinflammatorv 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.
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