The 2017 IUIS Phenotypic Classification for Primary Immunodeficiencies

Aziz Bousfiha*, Leïla Jeddane, Capucine Picard, Fatima Ailal, H. Bobby Gaspar, Waleed Al-Herz, Talal Chatila, Yanick J. Crow, Charlotte Cunningham-Rundles, Amos Etzioni, Jose Luis Franco, Steven M. Holland, Christoph Klein, Tomohiro Morio, Hans D. Ochs, Eric Oksenhendler, Jennifer Puck, Mimi L.K. Tang, Stuart G. Tangye, Troy R. TorgersonJean Laurent Casanova, Kathleen E. Sullivan

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Since the 1990s, the International Union of Immunological Societies (IUIS) PID expert committee (EC), now called Inborn Errors of Immunity Committee, has published every other year a classification of the inborn errors of immunity. This complete catalog serves as a reference for immunologists and researchers worldwide. However, it was unadapted for clinicians at the bedside. For those, the IUIS PID EC is now publishing a phenotypical classification since 2013, which proved to be more user-friendly. There are now 320 single-gene inborn errors of immunity underlying phenotypes as diverse as infection, malignancy, allergy, auto-immunity, and auto-inflammation. We herein propose the revised 2017 phenotypic classification, based on the accompanying 2017 IUIS Inborn Errors of Immunity Committee classification.

Original languageEnglish
Pages (from-to)129-143
Number of pages15
JournalJournal of Clinical Immunology
Volume38
Issue number1
Early online date11 Dec 2017
DOIs
Publication statusPublished - 1 Jan 2018

Keywords

  • Classification
  • Inborn errors of immunity
  • IUIS
  • Phenotypic
  • Primary immunodeficiencies

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