Human inborn errors of immunity to herpes viruses

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  • Emmanuelle Jouanguy, Institut National de la Santé et de la Recherche Médicale (INSERM), Paris Descartes University, The Rockefeller University
  • ,
  • Vivien Béziat, Institut National de la Santé et de la Recherche Médicale (INSERM), Paris Descartes University, The Rockefeller University
  • ,
  • Trine H. Mogensen
  • Jean Laurent Casanova
  • Stuart G. Tangye, Garvan Institute of Medical Research, University of New South Wales
  • ,
  • Shen Ying Zhang, Institut National de la Santé et de la Recherche Médicale (INSERM), Paris Descartes University, The Rockefeller University

Infections with any of the nine human herpes viruses (HHV) can be asymptomatic or life-threatening. The study of patients with severe diseases caused by HHVs, in the absence of overt acquired immunodeficiency, has led to the discovery or diagnosis of various inborn errors of immunity. The related inborn errors of adaptive immunity disrupt α/β T-cell rather than B-cell immunity. Affected patients typically develop HHV infections in the context of other infectious diseases. However, this is not always the case, as illustrated by inborn errors of SAP-dependent T-cell immunity to EBV-infected B cells. The related inborn errors of innate immunity disrupt leukocytes other than T and B cells, non-hematopoietic cells, or both. Patients typically develop only a single type of infection due to HHV, although, again, this is not always the case, as illustrated by inborn errors of TLR3 immunity resulting in HSV1 encephalitis in some patients and influenza pneumonitis in others. Most severe HHV infections in otherwise healthy patients remains unexplained. The forward human genetic dissection of isolated and syndromic HHV-driven illnesses will establish the molecular and cellular basis of protective immunity to HHVs, paving the way for novel diagnosis and management strategies.

Original languageEnglish
JournalCurrent Opinion in Immunology
Volume62
Pages (from-to)106-122
Number of pages17
ISSN0952-7915
DOIs
Publication statusPublished - Feb 2020

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