%0 Journal Article %T Autoinflammatory diseases in childhood %A Betul Sozeri %A Ozgur Kasapcopur %J Modern Research in Inflammation %P 7-18 %@ 2169-9690 %D 2014 %I Scientific Research Publishing %R 10.4236/mri.2014.31002 %X

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 1汕. In this review, it focuses on how recent advances have impacted hereditary autoinflammatory diseases.

%K Autoinflammatory Diseases %K Familial Mediterranean Fever %K Diagnosis %K Pathogenesis %K Treatment %U http://www.scirp.org/journal/PaperInformation.aspx?PaperID=42751