Encyclopedia of Medical Immunology

2014 Edition
| Editors: Ian R. Mackay, Noel R. Rose, Betty Diamond, Anne Davidson

Autoinflammatory Diseases

Reference work entry
First Online:
DOI: https://doi.org/10.1007/978-0-387-84828-0_414
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Synonyms

Autoinflammatory disorders; Hereditary periodic fever syndromes; Hereditary recurrent fever syndromes

Definition

Autoinflammatory diseases were originally described as a group of hereditary disorders characterized by recurrent, seemingly unprovoked inflammatory episodes without involvement of the adaptive immune response, i.e., absence of high-titer pathogenic autoantibodies or self-antigen-specific T lymphocytes (McDermott et al. 1999).

Its definition has recently been updated to encompass a larger group of inflammatory disorders mediated predominantly by the innate immune system and associated with a significant host predisposition, either as a Mendelian or a complex trait (Kastner et al. 2010). This definition does not rule out the contribution of adaptive immune response to the inflammatory process, but it aims to distinguish autoinflammatory diseases from autoimmune disorders, which are thought to develop through the effector responses of T and/or B cells directed...

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References

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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of Internal Medicine, Division of RheumatologyIstanbul Faculty of Medicine, Istanbul UniversityIstanbulTurkey