Typ-I-Interferonopathien

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• 作者：PD Dr. C. Günther ; F. Schmidt ; N. König ; M. A. Lee-Kirsch
• 关键词：Autoinflammation ; Autoimmunity ; Systemic lupus erythematosus ; Monogenic diseases ; Immune system
• 刊名：Zeitschrift f¨¹r Rheumatologie
• 出版年：2016
• 出版时间：March 2016
• 年：2016
• 卷：75
• 期：2
• 页码：134-140
• 全文大小：342 KB
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• 作者单位：PD Dr. C. Günther (1)
  F. Schmidt (1)
  N. König (2)
  M. A. Lee-Kirsch (2)
  
  1. Klinik für Dermatologie, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Fetscherstr. 74, 01307, Dresden, Deutschland
  2. Klinik für Kinder- und Jugendmedizin, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Deutschland
  
• 刊物主题：Internal Medicine; Rheumatology;
• 出版者：Springer Berlin Heidelberg
• ISSN：1435-1250

文摘

Type I interferons mediate immune defense against viral infections. The induction of type I interferons has stimulating and modulating effects on the innate and adaptive immune systems thereby reducing tolerance against self-antigens. Genetic defects that result in an inadequate activation of the type I interferon system can cause a group of inflammatory disorders, which are collectively referred to as type I interferonopathies. While the clinical spectrum of type I interferonopathies is broad and heterogeneous, neurological and cutaneous symptoms are the most frequent manifestations. Some clinical and genetic features of type I interferonopathies are shared by multifactorial diseases, such as systemic lupus erythematosus and systemic vasculitis. Advances in understanding the disease mechanisms underlying type I interferonopathies have pinpointed novel targets for therapeutic interventions.