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Autoinflammatory Syndromes

  • Living reference work entry
  • First Online:
Encyclopedia of Inflammatory Diseases

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Synonyms

Familial autoinflammatory diseases; Hereditary autoinflammatory diseases; Hereditary autoinflammatory syndromes; Hereditary periodic fevers; Inflammasomopathies; Periodic fever syndromes; Recurrent fever syndromes

Definition

Autoinflammatory disorders are a varied group of diseases characterized by recurrent episodes of seemingly unprovoked inflammation, unlike the inflammatory reactions triggered in the course of infections or during the immune response to autoantigens. These disorders are sometimes termed “inflammasomopathies,” stemming from the central role that interleukin (IL)-1 plays in their pathogenesis (Horvath et al. 2011; Wilson and Cassel 2010; see section on Pathophysiology, below).

Fever is a major fixture in these disorders. Indeed, most of our knowledge of these disorders comes from studies done to understand the pathogenesis of the inherited periodic fever syndromes. However, fever is not a universal trait of these conditions – several syndromes are...

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Authors and Affiliations

  1. Allergy & Immunology, Virginia Commonwealth University School of Medicine, 1112 E. Clay Street, McGuire Hall, Rm 4-110, Richmond, VA, 23298, USA

    Brant R. Ward

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  1. Brant R. Ward
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Correspondence to Brant R. Ward .

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  1. Inst of Clinical Pharmacology Haus 74/75, JW Goethe University Frankfurt Fraunhofer IME, Frankfurt, Germany

    Michael Parnham

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Ward, B.R. (2015). Autoinflammatory Syndromes. In: Parnham, M. (eds) Encyclopedia of Inflammatory Diseases. Birkhäuser, Basel. https://doi.org/10.1007/978-3-0348-0620-6_188-3

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  • DOI: https://doi.org/10.1007/978-3-0348-0620-6_188-3

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  • Online ISBN: 978-3-0348-0620-6

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