Modern Research in Inflammation

Volume 3, Issue 1 (February 2014)

ISSN Print: 2169-9682   ISSN Online: 2169-9690

Google-based Impact Factor: 0.43  Citations  

Autoinflammatory diseases in childhood

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DOI: 10.4236/mri.2014.31002    5,941 Downloads   10,088 Views  Citations


Autoinflammatory diseases are defined as recurrent attacks of systemic inflammation that are often unprovoked (or triggered by a minor event) related to a lack of adequate regulation of the innate immune system. Within the past decade, the list of autoinflammatory diseases has included cryopyrin-associated periodic syndromes, familial Mediterranean fever, mevalonate kinase deficiency, tumor necrosis factor receptor-associated periodic syndrome, hereditary pyogenic disorders, pediatric granulomatous autoinflammatory diseases, idiopathic febrile syndromes (systemic-onset juvenile idiopathic arthritis, PFAPA syndrome), complement dysregulation syndromes and Behcet’s disease. The hereditary autoinflammatory diseases are a group of Mendelian disorders characterized by seemingly unprovoked fever and localized inflammation. Autoinflammatory diseases can activate NOD-like receptors and inflammasome products including especially interleukin 1β. In this review, it focuses on how recent advances have impacted hereditary autoinflammatory diseases.

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Sozeri, B. and Kasapcopur, O. (2014) Autoinflammatory diseases in childhood. Modern Research in Inflammation, 3, 7-18. doi: 10.4236/mri.2014.31002.

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