Published in:
Open Access
01-11-2015 | Original Research
The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies
Authors:
Aziz Bousfiha, Leïla Jeddane, Waleed Al-Herz, Fatima Ailal, Jean‐Laurent Casanova, Talal Chatila, Mary Ellen Conley, Charlotte Cunningham‐Rundles, Amos Etzioni, Jose Luis Franco, H. Bobby Gaspar, Steven M. Holland, Christoph Klein, Shigeaki Nonoyama, Hans D. Ochs, Eric Oksenhendler, Capucine Picard, Jennifer M. Puck, Kathleen E. Sullivan, Mimi L. K. Tang
Published in:
Journal of Clinical Immunology
|
Issue 8/2015
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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.