The spectrum of monogenic autoinflammatory syndromes: understanding disease mechanisms and use of targeted therapies.

Abstract:

:Monogenic autoinflammatory diseases encompass a distinct and growing clinical entity of multisystem inflammatory diseases with known genetic defects in the innate immune system. The diseases present clinically with episodes of seemingly unprovoked inflammation (fever, rashes, and elevation of acute phase reactants). Understanding the genetics has led to discovery of new molecules involved in recognizing exogenous and endogenous danger signals, and the inflammatory response to these stimuli. These advances have furthered understanding of innate inflammatory pathways and spurred collaborative research in rheumatology and infectious diseases. The pivotal roles of interleukin (IL)-1beta in cryopyrin-associated periodic syndromes, tumor necrosis factor (TNF) in TNF receptor-associated periodic syndrome, and links to inflammatory cytokine dysregulation in other monogenic autoinflammatory diseases have resulted in effective therapies targeting proinflammatory cytokines IL-1beta and TNF and uncovered other new potential targets for anti-inflammatory therapies.

authors

Glaser RL,Goldbach-Mansky R

doi

10.1007/s11882-008-0047-1

subject

Has Abstract

pub_date

2008-07-01 00:00:00

pages

288-98

issue

4

eissn

1529-7322

issn

1534-6315

journal_volume

8

pub_type

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