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Autoinflammatory disorders

Chapter in book
Authors Per Wekell
Anders Fasth
Stefan Berg
Published in Aghamohammadi A, Rezaei N (eds). Clinical Cases in Primary Immunodeficiency Diseases 2012 .A Problem-Solving Approach
Pages 309 - 323
ISBN 9783642317842
Publisher Springer
Place of publication Berlin Heidelberg
Publication year 2012
Published at Institute of Clinical Sciences, Department of Pediatrics
Pages 309 - 323
Language en
Keywords autoinflammatory disorders, immunodeficiency
Subject categories Clinical immunology, Pediatrics


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].

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