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KMID : 0388220140210050228
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Journal of the Korean Rheumatism Association
2014 Volume.21 No. 5 p.228 ~ p.235
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Autoinflammatory Diseases
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Lee Sang-Heon
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Abstract
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Autoinflammatory diseases (AIDs) refer to a broad range of genetically mediated conditions characterized by recurrent at-tacks of systemic inflammation with typical manifestations of fever, rash, serositis, lymphadenopathy, and musculoskeletal symptoms. The discovery of genetic basis for these conditions have led to the understanding of novel intracellular receptors for infectious and noninfectious danger signals in innate immunity. Innate immunity has a key role in the development of AIDs, in contrast with autoimmune disease, which arise from problems in adaptive immunity. Advances in under-standing the molecular mechanisms of intracellular in-flammatory cascades have led to renewed interest in its role in the pathogenesis of more common non-genetic auto-inflammatory rheumatic conditions, such as Behcet’s disease, gouty arthritis, Adult onset Still’s diseases, and systemic onset juvenile arthritis. The characterization of cryopyrin (inflammasome) and its significant role in the activation of proinflammatory cytokines, such as IL-1β and TNF-α in the development of AIDs, has provided rational targets of anti-cy-tokine biologic treatment for some of these conditions.
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KEYWORD
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Inflammasome, cryopyrin, Innate immunity, Autoinflammatory rheumatic diseases
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