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J Clin Immunol. 2018 Jan;38(1):96-128. doi: 10.1007/s10875-017-0464-9. Epub 2017 Dec 11.

International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee Report on Inborn Errors of Immunity.

Author information

1
Center for the Study of Immunodeficiencies, Necker Hospital for Sick Children, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France.
2
Laboratory of Lymphocyte Activation and Susceptibility to EBV, INSERM UMR1163, Imagine Institute, Necker Hospital for Sick Children, Paris Descartes University, Paris, France.
3
UCL Great Ormond Street Institute of Child Health, London, UK.
4
Department of Pediatrics, Faculty of Medicine, Kuwait University, Kuwait City, Kuwait.
5
Laboratoire d'Immunologie Clinique, d'Inflammation et d'Allergy LICIA Clinical Immunology Unit, Casablanca Children's Hospital, Ibn Rochd Medical School, King Hassan II University, Casablanca, Morocco.
6
St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.
7
Howard Hughes Medical Institute, New York, NY, USA.
8
Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Imagine Institute, Necker Hospital for Sick Children, University Paris Descartes, Paris, France.
9
Pediatric Hematology-Immunology Unit, Necker Hospital for Sick Children APHP, Paris, France.
10
Division of Immunology, Children's Hospital Boston, Boston, MA, USA.
11
Laboratory of Neuroinflammation and Neurogenetics, Necker Branch, INSERM UMR1163, Paris Descartes University, Sorbonne-Paris-Cité, Institut Imagine, Paris, France.
12
Division of Evolution and Genomic Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK.
13
Departments of Medicine and Pediatrics, Mount Sinai School of Medicine, NewYork, NY, USA.
14
Ruth's Children's Hospital-Technion, Haifa, Israel.
15
Grupo de Inmunodeficiencias Primarias, Facultad de Medicina, Universidad de Antioquia UdeA, Medellin, Colombia.
16
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD, USA.
17
Dr von Hauner Children's Hospital, Ludwig-Maximilians-University Munich, Munich, Germany.
18
Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University (TMDU), Tokyo, Japan.
19
Department of Pediatrics, University of Washington and Seattle Children's Research Institute, Seattle, WA, USA.
20
Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, University Paris Diderot, Sorbonne Paris Cité, Paris, France.
21
Department of Pediatrics, University of California San Francisco and UCSF Benioff Children's Hospital, San Francisco, CA, USA.
22
Murdoch Children's Research Institute, Melbourne, VIC, Australia.
23
Department of Paediatrics, University of Melbourne, Melbourne, VIC, Australia.
24
Department of Allergy and Immunology, Royal Children's Hospital, Melbourne, Australia.
25
Immunology Division, Garvan Institute of Medical Research, Darlinghurst, NSW, Australia.
26
St Vincent's Clinical School, University of NSW, Sydney, Australia.
27
Division of Allergy Immunology, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, ARC 1216-I 3615 Civic Center Blvd, Philadelphia, PA, 19104, USA. sullivank@email.chop.edu.

Abstract

Beginning in 1970, a committee was constituted under the auspices of the World Health Organization (WHO) to catalog primary immunodeficiencies. Twenty years later, the International Union of Immunological Societies (IUIS) took the remit of this committee. The current report details the categorization and listing of 354 (as of February 2017) inborn errors of immunity. The growth and increasing complexity of the field have been impressive, encompassing an increasing variety of conditions, and the classification described here will serve as a critical reference for immunologists and researchers worldwide.

KEYWORDS:

IUIS; autoinflammatory disorders; immune dysregulation; primary immune deficiency

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