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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