The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies

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

Research output: Contribution to journalArticlepeer-review

173 Scopus citations


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.

Original languageEnglish
Pages (from-to)727-738
Number of pages12
JournalJournal of Clinical Immunology
Issue number8
StatePublished - 1 Nov 2015


  • IUIS PID expert committee
  • Primary immunodeficiencies
  • classification


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