Autoinflammatory diseases in childhood
Articles > Article · 2014
Authors: Betul Sozeri, Ozgur Kasapcopur
Modern Research in Inflammation, International, 2014, vol 03, pp 7-18
Summary
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 Behçet'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.