Journal
JOURNAL OF TRANSLATIONAL AUTOIMMUNITY
Volume 3, Issue -, Pages -Publisher
ELSEVIER
DOI: 10.1016/j.jtauto.2019.100031
Keywords
Autoinflammation; Autoinflammatory diseases; Periodic fevers
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Innate immune system represents the ancestral defense against infectious agents preserved along the evolution and species; it is phylogenetically older than the adaptive immune system, which exists only in the vertebrates. Cells with phagocytic activity such as neutrophils, macrophages, and natural killer (NK) cells play a key role in innate immunity. In 1999 Kastner a al. first introduced the term autoinflammation describing two diseases characterized by recurrent episodes of systemic inflammation without any identifiable infectious trigger: Familial Mediterranean Fever (FMF) and TNF Receptor Associated Periodic Syndrome (TRAPS). Autoinflammatory diseases (AIDs) are caused by self-directed inflammation due to an alteration of innate immunity leading to systemic inflammatory attacks typically in an on/off mode. In addition to inflammasomopathies, nuclear factor (NF)-kappa B-mediated disorders (also known as Rhelopathies) and type 1 interferonopathies are subjects of more recent studies. This review aims to provide an overview of the field with the most recent updates (see Most recent developments in.. paragraphs) and a description of the newly identified AIDs.
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