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Autoinflammatory Disorders

  • Per Wekell
  • Anders Fasth
  • Stefan Berg
Chapter

Abstract

Originally, autoinflammatory diseases were defined as conditions characterized by seemingly unprovoked episodes of inflammation, without high titres of autoantibodies or antigen-specific T-cells [1]. Recently, a new definition has been proposed in which autoinflammatory diseases are defined as “clinical disorders marked by abnormally increased inflammation, mediated predominantly by cells and molecules of the innate immune system, with a significant host predisposition [2].” These patients experience lifelong recurrent episodes of inflammation, associated with fever in most cases and other symptoms such as malaise, arthralgia, abdominal pain, and skin rash. Between the episodes, the patients often are free of symptoms [3].

Keywords

Familial Mediterranean Fever NLRP3 Inflammasome Primary Immunodeficiency Disease Autoinflammatory Disease Periodic Fever Syndrome 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  1. 1.Department of PediatricsUniversity of Gothenburg and NU-Hospital OrganizationUddevallaSweden
  2. 2.Department of PediatricsUniversity of Goteborg and the Queen Silvia Children’s HospitalGoteborgSweden

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