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The 2017 IUIS Phenotypic Classification for Primary Immunodeficiencies
- Bousfiha, Aziz;
- Jeddane, Leïla;
- Picard, Capucine;
- Ailal, Fatima;
- Bobby Gaspar, H;
- Al-Herz, Waleed;
- Chatila, Talal;
- Crow, Yanick J;
- Cunningham-Rundles, Charlotte;
- Etzioni, Amos;
- Franco, Jose Luis;
- Holland, Steven M;
- Klein, Christoph;
- Morio, Tomohiro;
- Ochs, Hans D;
- Oksenhendler, Eric;
- Puck, Jennifer;
- Tang, Mimi LK;
- Tangye, Stuart G;
- Torgerson, Troy R;
- Casanova, Jean-Laurent;
- Sullivan, Kathleen E
- et al.
Published Web Location
https://doi.org/10.1007/s10875-017-0465-8Abstract
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.
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