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Tunca, M., Akar, S., Onen, F., Ozdogan, H., Kasapcopur, O., Yalcinkaya, F., et al. (2005) Familial Mediterranean fever (FMF) in Turkey: Results of a nationwide multicenter study. Medicine (Baltimore), 84, 1-11. http://dx.doi.org/10.1097/01.md.0000152370.84628.0c

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• TITLE: Autoinflammatory diseases in childhood

  AUTHORS: Betul Sozeri, Ozgur Kasapcopur

  KEYWORDS: Autoinflammatory Diseases; Familial Mediterranean Fever; Diagnosis; Pathogenesis; Treatment

  JOURNAL NAME: Modern Research in Inflammation, Vol.3 No.1, February 11, 2014

  ABSTRACT: 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.