Document Detail


Treatment of autoinflammatory syndromes.
MedLine Citation:
PMID:  20966753     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
PURPOSE OF REVIEW: Inherited autoinflammatory diseases are experiments in nature in which mutations of proteins playing a pivotal role in the regulation of the innate immunity lead to unprovoked episodes of inflammation. The understanding of the molecular pathways involved in these disorders has shed a new light on the pattern of activation and maintenance of the inflammatory response and disclosed new molecular therapeutic targets. In this review, we outline the more recent novelties in the treatment of autoinflammatory diseases and their possible implications for some multifactorial pediatric conditions.
RECENT FINDINGS: Cryopyrin-associated periodic syndrome (CAPS) represents the prototype of autoinflammatory diseases. The study of the pathophysiological consequence of mutations of the cryopyrin gene (NLRP3) allowed the identification of the intracellular pathways thought to play a pivotal part in the activation and secretion of the potent inflammatory cytokine interleukin (IL)-1β. The dramatic effect of IL-1 blockade in CAPS opens new perspectives for the treatment of other inherited and multifactorial inflammatory disorders. A number of IL-1 blockers are now available on the market.
SUMMARY: Studies on the pathogenesis and treatment of inherited autoinflammatory diseases are also changing the approach to some multifactorial inflammatory conditions.
Authors:
Marco Gattorno; Alberto Martini
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Current opinion in pediatrics     Volume:  22     ISSN:  1531-698X     ISO Abbreviation:  Curr. Opin. Pediatr.     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2011-05-24     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9000850     Medline TA:  Curr Opin Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  771-8     Citation Subset:  IM    
Affiliation:
aUO Pediatria II, G. Gaslini Institute, Italy bDepartment of Pediatrics, University of Genova, Genova, Italy.
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