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Autoinflammatorische Erkrankungen

  • Eugen FeistEmail author
  • Jörg Henes
Living reference work entry
First Online:
Part of the Springer Reference Medizin book series (SRM)

Zusammenfassung

Im Gegensatz zu Autoimmunerkrankungen, bei denen ein Fehler bei Selbsterkennung und Immunabwehr im Vordergrund steht, führen bei den autoinflammatorischen Erkrankungen antigenunabhängige Entzündungsmechanismen zu einer unkontrollierten Überproduktion proinflammatorischer Zytokine. Autoinflammatorische Erkrankungen sind typischerweise durch Fieber gekennzeichnet (Fiebersyndrome), das bei serologischer Entzündungsaktivität mit weiteren Organmanifestationen, häufig unter Einbeziehung der Haut und des muskuloskelettalen Systems, einhergehen kann. Das Spektrum ist vielfältig, wobei regelmäßig neue, oft sehr seltene Krankheitsbilder beschrieben werden. Die meisten dieser Erkrankungen beginnen bereits im Kindesalter, teilweise können erste Erkrankungszeichen aber auch erst im Erwachsenenalter auftreten. Dieses Kapitel geht auf folgende Erkrankungen ein: Still-Syndrom des Erwachsenen, Schnitzler-Syndrom, PFAPA-Syndrom, Familiäres Mittelmeerfieber (FMF), Cryopyrin-assoziierte periodische Syndrome (CAPS), Tumornekrosefaktor-alpha-Rezeptor-assoziiertes periodisches Syndrom (TRAPS) und Hyper-IgD-Syndrom.

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Copyright information

© Springer-Verlag GmbH Deutschland, ein Teil von Springer Nature 2019

Authors and Affiliations

  1. 1.Klinik für Rheumatologie und Klinische ImmunologieCharité – Universitätsmedizin BerlinBerlinDeutschland
  2. 2.Medizinische Universitätsklinik Abt. II (Onkologie, Hämatologie, Rheumatologie, Immunologie, Pulmonologie)Universitätsklinikum TübingenTübingenDeutschland

Section editors and affiliations

  • Elisabeth Märker-Hermann
    • 1
  1. 1.Klinik Innere Medizin IV, Rheumatologie, klinische Immunologie und NephrologieHelios Dr. Horst Schmidt Kliniken WiesbadenWiesbadenDeutschland

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