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The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies

Authors :
H. Bobby Gaspar
Waleed Al-Herz
Talal A. Chatila
Jean-Laurent Casanova
José Luis Franco
Mimi L.K. Tang
Capucine Picard
Christoph Klein
Amos Etzioni
Charlotte Cunningham-Rundles
Shigeaki Nonoyama
Steven M. Holland
Leila Jeddane
Kathleen E. Sullivan
Jennifer M. Puck
Mary Ellen Conley
Hans D. Ochs
Aziz Bousfiha
Fatima Ailal
Eric Oksenhendler
Source :
Journal of Clinical Immunology
Publication Year :
2015
Publisher :
Springer Science and Business Media LLC, 2015.

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.

Details

ISSN :
15732592 and 02719142
Volume :
35
Database :
OpenAIRE
Journal :
Journal of Clinical Immunology
Accession number :
edsair.doi.dedup.....8f8df2d14dfaa617336b22506bff7078
Full Text :
https://doi.org/10.1007/s10875-015-0198-5