October 30, 2015

The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies


 2015 Oct 7. [Epub ahead of print]

Open Access

  • Aziz Bousfiha, 
  • Leïla Jeddane
  • , Waleed AlHerz
  • , Fatima Ailal
  • , JeanLaurent Casanova
  • ,Talal Chatila
  • , Mary Ellen Conley
  • , Charlotte Cunningham‐Rundles
  • , Amos Etzioni
  •  and 11 more

    • Abstract

    There are now nearly 300 single-gene inborn errors of immunity underlying phenotypes as diverse as infection, malignancy, allergy, auto-immunity, and auto-inflammation. For each of these five categories, a growing variety of phenotypes are ascribed to Primary Immunodeficiency Diseases (PID), making PIDs a rapidly expanding field of medicine.
    The International Union of Immunological Societies (IUIS) PID expert committee (EC) has published every other year a classification of these disorders into tables, defined by shared pathogenesis and/or clinical consequences. In 2013, the IUIS committee also proposed a more user-friendly, phenotypic classification, based on the selection of key phenotypes at the bedside. We herein propose the revised figures, based on the accompanying 2015 IUIS PID EC classification.

    Keywords

    Primary immunodeficiencies classification IUIS PID expert committee

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