The 2017 IUIS Phenotypic Classification for Primary Immunodeficiencies

Affiliations

01 January 2018

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doi: 10.1007/s10875-017-0465-8


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.

Keywords: Classification; IUIS; Inborn errors of immunity; Phenotypic; Primary immunodeficiencies.

Conflict of interest statement

The authors declare that they have no conflict of interest.


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