title: The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies.
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.
authors.affiliation: Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco. profbousfiha@gmail.com.
authors.forename: Aziz
authors.initials: A
authors.lastname: Bousfiha
authors.affiliation: Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco.
authors.forename: Leïla
authors.initials: L
authors.lastname: Jeddane
authors.affiliation: Department of Pediatrics, Faculty of Medicine Kuwait University, Jabriya, Kuwait.|Allergy and Clinical Immunology Unit, Department of Pediatrics, Al-Sabah Hospital, Kuwait City, Kuwait.
authors.forename: Waleed
authors.initials: W
authors.lastname: Al-Herz
authors.affiliation: Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco.
authors.forename: Fatima
authors.initials: F
authors.lastname: Ailal
authors.affiliation: St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.|Howard Hughes Medical Institute, New York, NY, USA.|Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Necker Hospital for Sick Children, Paris, France.|Imagine Institute, University Paris Descartes, Paris, France.|Pediatric Hematology & Immunology Unit, Necker Hospital for Sick Children, Paris, France.
authors.forename: Jean-Laurent
authors.initials: JL
authors.lastname: Casanova
authors.affiliation: Division of Immunology, Children's Hospital Boston, Boston, MA, USA.
authors.forename: Talal
authors.initials: T
authors.lastname: Chatila
authors.affiliation: St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.
authors.forename: Mary Ellen
authors.initials: ME
authors.lastname: Conley
authors.affiliation: Department of Medicine and Pediatrics, Mount Sinai School of Medicine, New York, NY, USA.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Cunningham-Rundles
authors.affiliation: Meyer Children's Hospital-Technion, Haifa, Israel.
authors.forename: Amos
authors.initials: A
authors.lastname: Etzioni
authors.affiliation: Group of Primary Immunodeficiencies, University of Antioquia, Medellin, Colombia.
authors.forename: Jose Luis
authors.initials: JL
authors.lastname: Franco
authors.affiliation: UCL Institute of Child Health, London, UK.
authors.forename: H Bobby
authors.initials: HB
authors.lastname: Gaspar
authors.affiliation: Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD, USA.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
authors.affiliation: Dr von Hauner Children's Hospital, Ludwig-Maximilians University Munich, Munich, Germany.
authors.forename: Christoph
authors.initials: C
authors.lastname: Klein
authors.affiliation: Department of Pediatrics, National Defense Medical College, Saitama, Japan.
authors.forename: Shigeaki
authors.initials: S
authors.lastname: Nonoyama
authors.affiliation: Department of Pediatrics, University of Washington and Seattle Children's Research Institute, Seattle, WA, USA.
authors.forename: Hans D
authors.initials: HD
authors.lastname: Ochs
authors.affiliation: Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France.|Université Paris Diderot, Sorbonne Paris Cité, Paris, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Oksenhendler
authors.affiliation: Howard Hughes Medical Institute, New York, NY, USA.|Centre d'étude des déficits immunitaires (CEDI), Hôpital Necker-Enfants Malades, AP-HP, Paris, France.
authors.forename: Capucine
authors.initials: C
authors.lastname: Picard
authors.affiliation: Department of Pediatrics, University of California San Francisco and UCSF Benioff Children's Hospital, San Francisco, CA, USA.
authors.forename: Jennifer M
authors.initials: JM
authors.lastname: Puck
authors.affiliation: Division of Allergy Immunology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Kathleen E
authors.initials: KE
authors.lastname: Sullivan
authors.affiliation: Murdoch Childrens Research Institute, Melbourne, VIC, Australia.|Department of Paediatrics, University of Melbourne, Melbourne, VIC, Australia.|Department of Allergy and Immunology, Royal Children's Hospital, Melbourne, VIC, Australia.
authors.forename: Mimi L K
authors.initials: ML
authors.lastname: Tang
mesh_terms: D015551:Autoimmunity; D005104:Expert Testimony; D006801:Humans; D006967:Hypersensitivity / Q000276:immunology*; D007109:Immunity / Q000235:genetics; D007153:Immunologic Deficiency Syndromes / Q000145:classification / Q000276:immunology*; D007239:Infections / Q000276:immunology*; D007249:Inflammation / Q000276:immunology*; D009369:Neoplasms / Q000276:immunology*; D010641:Phenotype
keywords: IUIS PID expert committee; Primary immunodeficiencies; classification