Autoinflammatory Diseases.
10.4078/jrd.2014.21.5.228
- Author:
Sang Heon LEE
1
Author Information
1. Division of Rheumatology, Konkuk Universtiy School of Medicine, Seoul, Korea. shlee@kuh.ac.kr
- Publication Type:Review
- Keywords:
Inflammasome;
cryopyrin;
Innate immunity;
Autoinflammatory rheumatic diseases
- MeSH:
Adaptive Immunity;
Adult;
Arthritis, Gouty;
Arthritis, Juvenile;
Autoimmune Diseases;
Cytokines;
Exanthema;
Fever;
Humans;
Immunity, Innate;
Inflammation;
Lymphatic Diseases;
Serositis;
Tumor Necrosis Factor-alpha
- From:Journal of Rheumatic Diseases
2014;21(5):228-235
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Autoinflammatory diseases (AIDs) refer to a broad range of genetically mediated conditions characterized by recurrent attacks 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 understanding the molecular mechanisms of intracellular inflammatory cascades have led to renewed interest in its role in the pathogenesis of more common non-genetic autoinflammatory 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-1beta and TNF-alpha in the development of AIDs, has provided rational targets of anti-cytokine biologic treatment for some of these conditions.