REVIEW - The spectrum of autoinflammatory diseases: recent bench to bedside observations

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Medscape.com

Posted 02/20/2008

G. ;a Raphaela Goldbach-Mansky

The Spectrum of Autoinflammatory Diseases: Recent Bench to Bedside Observations

Abstract

Purpose of review: The autoinflammatory diseases are a group of

conditions that include the hereditary fever syndromes, and result

from upregulated innate immune responses. The discovery of the genetic

basis for these conditions led to the description of novel

intracellular receptors for infectious and noninfectious danger

signals. This article focuses on recent progress in our understanding

of autoinflammatory syndromes, and how insights into these conditions

have triggered the exploration of the role of innate immunity in

common rheumatologic diseases.

Recent findings: New models for the pathogenesis of several

autoinflammatory syndromes have been proposed, including the role of

pyrin and cryopyrin in regulating inflammation. Robust evidence has

emerged that IL-1â oversecretion is pivotal in cryopyrin-associated

periodic syndromes, and that IL-1 inhibition ameliorates the clinical

features of these syndromes. Monosodium urate crystals stimulate IL-1â

secretion via cryopyrin, which led to the addition of gout to the

spectrum of autoinflammatory diseases.

Summary: Advances in our understanding of the autoinflammatory

diseases have led to renewed interest in the innate immune system, and

its role in the pathogenesis of more common rheumatic diseases.

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http://www.medscape.com/viewarticle/568454

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