Type I Interferonopathies: From Pathophysiology to Clinical Expression

  • Christina Maria Flessa
  • Evangelia Argiriou
  • Clio P. Mavragani


Type I interferonopathies are a diverse group of monogenic diseases which hallmarked the type I interferon (IFN) pathway activation. Abnormal accumulation of endogenous nucleic acids, excessive sensitivity or activity of DNA/RNA sensors, and the dysregulation of type I IFN pathway have been identified as the main contributors to excessive type I IFN signaling in this setting. Chilblain-like lesions, central nervous system calcifications, interstitial lung disease, and growth retardation are among the most common features shared by the majority of type I interferonopathies. Targeting of type I IFN signaling seems to hold a promising therapeutic role.


Interferonopathies Type I interferon Toll-like receptors Aicardi-Goutieres syndrome SAVI STING Chilblain Endosomal receptors Cytosolic receptors 


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Christina Maria Flessa
    • 1
  • Evangelia Argiriou
    • 1
  • Clio P. Mavragani
    • 1
  1. 1.Department of Physiology, School of MedicineNational and Kapodistrian University of AthensAthensGreece

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