The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies.
Identifieur interne : 002613 ( PubMed/Checkpoint ); précédent : 002612; suivant : 002614The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies.
Auteurs : Aziz Bousfiha [Maroc] ; Leïla Jeddane [Maroc] ; Waleed Al-Herz [Koweït] ; Fatima Ailal [Maroc] ; Jean-Laurent Casanova [États-Unis] ; Talal Chatila [États-Unis] ; Mary Ellen Conley [États-Unis] ; Charlotte Cunningham-Rundles [États-Unis] ; Amos Etzioni [Israël] ; Jose Luis Franco [Colombie] ; H Bobby Gaspar [Royaume-Uni] ; Steven M. Holland [États-Unis] ; Christoph Klein [Allemagne] ; Shigeaki Nonoyama [Japon] ; Hans D. Ochs [États-Unis] ; Eric Oksenhendler [France] ; Capucine Picard [États-Unis] ; Jennifer M. Puck [États-Unis] ; Kathleen E. Sullivan [États-Unis] ; Mimi L K. Tang [Australie]Source :
- Journal of clinical immunology [ 1573-2592 ] ; 2015.
Descripteurs français
- KwdFr :
- MESH :
- génétique : Immunité.
- immunologie : Déficits immunitaires, Hypersensibilité, Infection, Inflammation, Tumeurs.
- Auto-immunité, Déficits immunitaires, Expertise, Humains, Phénotype.
English descriptors
- KwdEn :
- MESH :
- classification : Immunologic Deficiency Syndromes.
- genetics : Immunity.
- immunology : Hypersensitivity, Immunologic Deficiency Syndromes, Infection, Inflammation, Neoplasms.
- Autoimmunity, Expert Testimony, Humans, Phenotype.
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.
DOI: 10.1007/s10875-015-0198-5
PubMed: 26445875
Affiliations:
- Allemagne, Australie, Colombie, France, Israël, Japon, Koweït, Maroc, Royaume-Uni, États-Unis
- Angleterre, Bavière, Californie, District de Haute-Bavière, Grand Londres, Maryland, Massachusetts, Pennsylvanie, Washington (État), État de New York, Île-de-France
- Londres, Munich, Paris
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Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco.</nlm:affiliation><country xml:lang="fr">Maroc</country><wicri:regionArea>Clinical Immunology Unit, A. 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Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco.</nlm:affiliation><country xml:lang="fr">Maroc</country><wicri:regionArea>Clinical Immunology Unit, A. 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Holland</name><affiliation wicri:level="2"><nlm:affiliation>Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD</wicri:regionArea><placeName><region type="state">Maryland</region></placeName></affiliation></author><author><name sortKey="Klein, Christoph" sort="Klein, Christoph" uniqKey="Klein C" first="Christoph" last="Klein">Christoph Klein</name><affiliation wicri:level="3"><nlm:affiliation>Dr von Hauner Children's Hospital, Ludwig-Maximilians University Munich, Munich, Germany.</nlm:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Dr von Hauner Children's Hospital, Ludwig-Maximilians University Munich, Munich</wicri:regionArea><placeName><region type="land" nuts="1">Bavière</region><region type="district" nuts="2">District de Haute-Bavière</region><settlement type="city">Munich</settlement></placeName></affiliation></author><author><name sortKey="Nonoyama, Shigeaki" sort="Nonoyama, Shigeaki" uniqKey="Nonoyama S" first="Shigeaki" last="Nonoyama">Shigeaki Nonoyama</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pediatrics, National Defense Medical College, Saitama, Japan.</nlm:affiliation><country xml:lang="fr">Japon</country><wicri:regionArea>Department of Pediatrics, National Defense Medical College, Saitama</wicri:regionArea><wicri:noRegion>Saitama</wicri:noRegion></affiliation></author><author><name sortKey="Ochs, Hans D" sort="Ochs, Hans D" uniqKey="Ochs H" first="Hans D" last="Ochs">Hans D. Ochs</name><affiliation wicri:level="2"><nlm:affiliation>Department of Pediatrics, University of Washington and Seattle Children's Research Institute, Seattle, WA, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Pediatrics, University of Washington and Seattle Children's Research Institute, Seattle, WA</wicri:regionArea><placeName><region type="state">Washington (État)</region></placeName></affiliation></author><author><name sortKey="Oksenhendler, Eric" sort="Oksenhendler, Eric" uniqKey="Oksenhendler E" first="Eric" last="Oksenhendler">Eric Oksenhendler</name><affiliation wicri:level="3"><nlm:affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France.</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name><affiliation wicri:level="2"><nlm:affiliation>Howard Hughes Medical Institute, New York, NY, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Howard Hughes Medical Institute, New York, NY</wicri:regionArea><placeName><region type="state">État de New York</region></placeName></affiliation></author><author><name sortKey="Puck, Jennifer M" sort="Puck, Jennifer M" uniqKey="Puck J" first="Jennifer M" last="Puck">Jennifer M. Puck</name><affiliation wicri:level="2"><nlm:affiliation>Department of Pediatrics, University of California San Francisco and UCSF Benioff Children's Hospital, San Francisco, CA, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Pediatrics, University of California San Francisco and UCSF Benioff Children's Hospital, San Francisco, CA</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Sullivan, Kathleen E" sort="Sullivan, Kathleen E" uniqKey="Sullivan K" first="Kathleen E" last="Sullivan">Kathleen E. Sullivan</name><affiliation wicri:level="2"><nlm:affiliation>Division of Allergy Immunology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Division of Allergy Immunology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA</wicri:regionArea><placeName><region type="state">Pennsylvanie</region></placeName></affiliation></author><author><name sortKey="Tang, Mimi L K" sort="Tang, Mimi L K" uniqKey="Tang M" first="Mimi L K" last="Tang">Mimi L K. Tang</name><affiliation wicri:level="1"><nlm:affiliation>Murdoch Childrens Research Institute, Melbourne, VIC, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Murdoch Childrens Research Institute, Melbourne, VIC</wicri:regionArea><wicri:noRegion>VIC</wicri:noRegion></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2015">2015</date><idno type="RBID">pubmed:26445875</idno><idno type="pmid">26445875</idno><idno type="doi">10.1007/s10875-015-0198-5</idno><idno type="wicri:Area/PubMed/Corpus">002773</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">002773</idno><idno type="wicri:Area/PubMed/Curation">002704</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">002704</idno><idno type="wicri:Area/PubMed/Checkpoint">002704</idno><idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">002704</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies.</title><author><name sortKey="Bousfiha, Aziz" sort="Bousfiha, Aziz" uniqKey="Bousfiha A" first="Aziz" last="Bousfiha">Aziz Bousfiha</name><affiliation wicri:level="1"><nlm:affiliation>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco. profbousfiha@gmail.com.</nlm:affiliation><country xml:lang="fr">Maroc</country><wicri:regionArea>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca</wicri:regionArea><wicri:noRegion>Casablanca</wicri:noRegion></affiliation></author><author><name sortKey="Jeddane, Leila" sort="Jeddane, Leila" uniqKey="Jeddane L" first="Leïla" last="Jeddane">Leïla Jeddane</name><affiliation wicri:level="1"><nlm:affiliation>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco.</nlm:affiliation><country xml:lang="fr">Maroc</country><wicri:regionArea>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca</wicri:regionArea><wicri:noRegion>Casablanca</wicri:noRegion></affiliation></author><author><name sortKey="Al Herz, Waleed" sort="Al Herz, Waleed" uniqKey="Al Herz W" first="Waleed" last="Al-Herz">Waleed Al-Herz</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pediatrics, Faculty of Medicine Kuwait University, Jabriya, Kuwait.</nlm:affiliation><country xml:lang="fr">Koweït</country><wicri:regionArea>Department of Pediatrics, Faculty of Medicine Kuwait University, Jabriya</wicri:regionArea><wicri:noRegion>Jabriya</wicri:noRegion></affiliation></author><author><name sortKey="Ailal, Fatima" sort="Ailal, Fatima" uniqKey="Ailal F" first="Fatima" last="Ailal">Fatima Ailal</name><affiliation wicri:level="1"><nlm:affiliation>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco.</nlm:affiliation><country xml:lang="fr">Maroc</country><wicri:regionArea>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca</wicri:regionArea><wicri:noRegion>Casablanca</wicri:noRegion></affiliation></author><author><name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name><affiliation wicri:level="2"><nlm:affiliation>St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY</wicri:regionArea><placeName><region type="state">État de New York</region></placeName></affiliation></author><author><name sortKey="Chatila, Talal" sort="Chatila, Talal" uniqKey="Chatila T" first="Talal" last="Chatila">Talal Chatila</name><affiliation wicri:level="2"><nlm:affiliation>Division of Immunology, Children's Hospital Boston, Boston, MA, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Division of Immunology, Children's Hospital Boston, Boston, MA</wicri:regionArea><placeName><region type="state">Massachusetts</region></placeName></affiliation></author><author><name sortKey="Conley, Mary Ellen" sort="Conley, Mary Ellen" uniqKey="Conley M" first="Mary Ellen" last="Conley">Mary Ellen Conley</name><affiliation wicri:level="2"><nlm:affiliation>St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY</wicri:regionArea><placeName><region type="state">État de New York</region></placeName></affiliation></author><author><name sortKey="Cunningham Rundles, Charlotte" sort="Cunningham Rundles, Charlotte" uniqKey="Cunningham Rundles C" first="Charlotte" last="Cunningham-Rundles">Charlotte Cunningham-Rundles</name><affiliation wicri:level="2"><nlm:affiliation>Department of Medicine and Pediatrics, Mount Sinai School of Medicine, New York, NY, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Medicine and Pediatrics, Mount Sinai School of Medicine, New York, NY</wicri:regionArea><placeName><region type="state">État de New York</region></placeName></affiliation></author><author><name sortKey="Etzioni, Amos" sort="Etzioni, Amos" uniqKey="Etzioni A" first="Amos" last="Etzioni">Amos Etzioni</name><affiliation wicri:level="1"><nlm:affiliation>Meyer Children's Hospital-Technion, Haifa, Israel.</nlm:affiliation><country xml:lang="fr">Israël</country><wicri:regionArea>Meyer Children's Hospital-Technion, Haifa</wicri:regionArea><wicri:noRegion>Haifa</wicri:noRegion></affiliation></author><author><name sortKey="Franco, Jose Luis" sort="Franco, Jose Luis" uniqKey="Franco J" first="Jose Luis" last="Franco">Jose Luis Franco</name><affiliation wicri:level="1"><nlm:affiliation>Group of Primary Immunodeficiencies, University of Antioquia, Medellin, Colombia.</nlm:affiliation><country xml:lang="fr">Colombie</country><wicri:regionArea>Group of Primary Immunodeficiencies, University of Antioquia, Medellin</wicri:regionArea><wicri:noRegion>Medellin</wicri:noRegion></affiliation></author><author><name sortKey="Gaspar, H Bobby" sort="Gaspar, H Bobby" uniqKey="Gaspar H" first="H Bobby" last="Gaspar">H Bobby Gaspar</name><affiliation wicri:level="3"><nlm:affiliation>UCL Institute of Child Health, London, UK.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>UCL Institute of Child Health, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Holland, Steven M" sort="Holland, Steven M" uniqKey="Holland S" first="Steven M" last="Holland">Steven M. Holland</name><affiliation wicri:level="2"><nlm:affiliation>Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD</wicri:regionArea><placeName><region type="state">Maryland</region></placeName></affiliation></author><author><name sortKey="Klein, Christoph" sort="Klein, Christoph" uniqKey="Klein C" first="Christoph" last="Klein">Christoph Klein</name><affiliation wicri:level="3"><nlm:affiliation>Dr von Hauner Children's Hospital, Ludwig-Maximilians University Munich, Munich, Germany.</nlm:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Dr von Hauner Children's Hospital, Ludwig-Maximilians University Munich, Munich</wicri:regionArea><placeName><region type="land" nuts="1">Bavière</region><region type="district" nuts="2">District de Haute-Bavière</region><settlement type="city">Munich</settlement></placeName></affiliation></author><author><name sortKey="Nonoyama, Shigeaki" sort="Nonoyama, Shigeaki" uniqKey="Nonoyama S" first="Shigeaki" last="Nonoyama">Shigeaki Nonoyama</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pediatrics, National Defense Medical College, Saitama, Japan.</nlm:affiliation><country xml:lang="fr">Japon</country><wicri:regionArea>Department of Pediatrics, National Defense Medical College, Saitama</wicri:regionArea><wicri:noRegion>Saitama</wicri:noRegion></affiliation></author><author><name sortKey="Ochs, Hans D" sort="Ochs, Hans D" uniqKey="Ochs H" first="Hans D" last="Ochs">Hans D. Ochs</name><affiliation wicri:level="2"><nlm:affiliation>Department of Pediatrics, University of Washington and Seattle Children's Research Institute, Seattle, WA, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Pediatrics, University of Washington and Seattle Children's Research Institute, Seattle, WA</wicri:regionArea><placeName><region type="state">Washington (État)</region></placeName></affiliation></author><author><name sortKey="Oksenhendler, Eric" sort="Oksenhendler, Eric" uniqKey="Oksenhendler E" first="Eric" last="Oksenhendler">Eric Oksenhendler</name><affiliation wicri:level="3"><nlm:affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France.</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name><affiliation wicri:level="2"><nlm:affiliation>Howard Hughes Medical Institute, New York, NY, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Howard Hughes Medical Institute, New York, NY</wicri:regionArea><placeName><region type="state">État de New York</region></placeName></affiliation></author><author><name sortKey="Puck, Jennifer M" sort="Puck, Jennifer M" uniqKey="Puck J" first="Jennifer M" last="Puck">Jennifer M. Puck</name><affiliation wicri:level="2"><nlm:affiliation>Department of Pediatrics, University of California San Francisco and UCSF Benioff Children's Hospital, San Francisco, CA, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Pediatrics, University of California San Francisco and UCSF Benioff Children's Hospital, San Francisco, CA</wicri:regionArea><placeName><region type="state">Californie</region></placeName></affiliation></author><author><name sortKey="Sullivan, Kathleen E" sort="Sullivan, Kathleen E" uniqKey="Sullivan K" first="Kathleen E" last="Sullivan">Kathleen E. Sullivan</name><affiliation wicri:level="2"><nlm:affiliation>Division of Allergy Immunology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Division of Allergy Immunology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA</wicri:regionArea><placeName><region type="state">Pennsylvanie</region></placeName></affiliation></author><author><name sortKey="Tang, Mimi L K" sort="Tang, Mimi L K" uniqKey="Tang M" first="Mimi L K" last="Tang">Mimi L K. Tang</name><affiliation wicri:level="1"><nlm:affiliation>Murdoch Childrens Research Institute, Melbourne, VIC, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Murdoch Childrens Research Institute, Melbourne, VIC</wicri:regionArea><wicri:noRegion>VIC</wicri:noRegion></affiliation></author></analytic><series><title level="j">Journal of clinical immunology</title><idno type="eISSN">1573-2592</idno><imprint><date when="2015" type="published">2015</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Autoimmunity</term><term>Expert Testimony</term><term>Humans</term><term>Hypersensitivity (immunology)</term><term>Immunity (genetics)</term><term>Immunologic Deficiency Syndromes (classification)</term><term>Immunologic Deficiency Syndromes (immunology)</term><term>Infection (immunology)</term><term>Inflammation (immunology)</term><term>Neoplasms (immunology)</term><term>Phenotype</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Auto-immunité</term><term>Déficits immunitaires ()</term><term>Déficits immunitaires (immunologie)</term><term>Expertise</term><term>Humains</term><term>Hypersensibilité (immunologie)</term><term>Immunité (génétique)</term><term>Infection (immunologie)</term><term>Inflammation (immunologie)</term><term>Phénotype</term><term>Tumeurs (immunologie)</term></keywords><keywords scheme="MESH" qualifier="classification" xml:lang="en"><term>Immunologic Deficiency Syndromes</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Immunity</term></keywords><keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Immunité</term></keywords><keywords scheme="MESH" qualifier="immunologie" xml:lang="fr"><term>Déficits immunitaires</term><term>Hypersensibilité</term><term>Infection</term><term>Inflammation</term><term>Tumeurs</term></keywords><keywords scheme="MESH" qualifier="immunology" xml:lang="en"><term>Hypersensitivity</term><term>Immunologic Deficiency Syndromes</term><term>Infection</term><term>Inflammation</term><term>Neoplasms</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Autoimmunity</term><term>Expert Testimony</term><term>Humans</term><term>Phenotype</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Auto-immunité</term><term>Déficits immunitaires</term><term>Expertise</term><term>Humains</term><term>Phénotype</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">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.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">26445875</PMID><DateCreated><Year>2015</Year><Month>11</Month><Day>26</Day></DateCreated><DateCompleted><Year>2016</Year><Month>09</Month><Day>05</Day></DateCompleted><DateRevised><Year>2017</Year><Month>02</Month><Day>20</Day></DateRevised><Article PubModel="Print-Electronic"><Journal><ISSN IssnType="Electronic">1573-2592</ISSN><JournalIssue CitedMedium="Internet"><Volume>35</Volume><Issue>8</Issue><PubDate><Year>2015</Year><Month>Nov</Month></PubDate></JournalIssue><Title>Journal of clinical immunology</Title><ISOAbbreviation>J. Clin. Immunol.</ISOAbbreviation></Journal><ArticleTitle>The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies.</ArticleTitle><Pagination><MedlinePgn>727-38</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1007/s10875-015-0198-5</ELocationID><Abstract><AbstractText>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.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Bousfiha</LastName><ForeName>Aziz</ForeName><Initials>A</Initials><AffiliationInfo><Affiliation>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco. profbousfiha@gmail.com.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Jeddane</LastName><ForeName>Leïla</ForeName><Initials>L</Initials><AffiliationInfo><Affiliation>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Al-Herz</LastName><ForeName>Waleed</ForeName><Initials>W</Initials><AffiliationInfo><Affiliation>Department of Pediatrics, Faculty of Medicine Kuwait University, Jabriya, Kuwait.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Allergy and Clinical Immunology Unit, Department of Pediatrics, Al-Sabah Hospital, Kuwait City, Kuwait.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Ailal</LastName><ForeName>Fatima</ForeName><Initials>F</Initials><AffiliationInfo><Affiliation>Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Casanova</LastName><ForeName>Jean-Laurent</ForeName><Initials>JL</Initials><AffiliationInfo><Affiliation>St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Howard Hughes Medical Institute, New York, NY, USA.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR1163, Necker Hospital for Sick Children, Paris, France.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Imagine Institute, University Paris Descartes, Paris, France.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Pediatric Hematology & Immunology Unit, Necker Hospital for Sick Children, Paris, France.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Chatila</LastName><ForeName>Talal</ForeName><Initials>T</Initials><AffiliationInfo><Affiliation>Division of Immunology, Children's Hospital Boston, Boston, MA, USA.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Conley</LastName><ForeName>Mary Ellen</ForeName><Initials>ME</Initials><AffiliationInfo><Affiliation>St. Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, New York, NY, USA.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Cunningham-Rundles</LastName><ForeName>Charlotte</ForeName><Initials>C</Initials><AffiliationInfo><Affiliation>Department of Medicine and Pediatrics, Mount Sinai School of Medicine, New York, NY, USA.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Etzioni</LastName><ForeName>Amos</ForeName><Initials>A</Initials><AffiliationInfo><Affiliation>Meyer Children's Hospital-Technion, Haifa, Israel.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Franco</LastName><ForeName>Jose Luis</ForeName><Initials>JL</Initials><AffiliationInfo><Affiliation>Group of Primary Immunodeficiencies, University of Antioquia, Medellin, Colombia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Gaspar</LastName><ForeName>H Bobby</ForeName><Initials>HB</Initials><AffiliationInfo><Affiliation>UCL Institute of Child Health, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Holland</LastName><ForeName>Steven M</ForeName><Initials>SM</Initials><AffiliationInfo><Affiliation>Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Bethesda, MD, USA.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Klein</LastName><ForeName>Christoph</ForeName><Initials>C</Initials><AffiliationInfo><Affiliation>Dr von Hauner Children's Hospital, Ludwig-Maximilians University Munich, Munich, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Nonoyama</LastName><ForeName>Shigeaki</ForeName><Initials>S</Initials><AffiliationInfo><Affiliation>Department of Pediatrics, National Defense Medical College, Saitama, Japan.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Ochs</LastName><ForeName>Hans D</ForeName><Initials>HD</Initials><AffiliationInfo><Affiliation>Department of Pediatrics, University of Washington and Seattle Children's Research Institute, Seattle, WA, USA.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Oksenhendler</LastName><ForeName>Eric</ForeName><Initials>E</Initials><AffiliationInfo><Affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Université Paris Diderot, Sorbonne Paris Cité, Paris, France.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Picard</LastName><ForeName>Capucine</ForeName><Initials>C</Initials><AffiliationInfo><Affiliation>Howard Hughes Medical Institute, New York, NY, USA.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Centre d'étude des déficits immunitaires (CEDI), Hôpital Necker-Enfants Malades, AP-HP, Paris, France.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Puck</LastName><ForeName>Jennifer M</ForeName><Initials>JM</Initials><AffiliationInfo><Affiliation>Department of Pediatrics, University of California San Francisco and UCSF Benioff Children's Hospital, San Francisco, CA, USA.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Sullivan</LastName><ForeName>Kathleen E</ForeName><Initials>KE</Initials><AffiliationInfo><Affiliation>Division of Allergy Immunology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Tang</LastName><ForeName>Mimi L K</ForeName><Initials>ML</Initials><AffiliationInfo><Affiliation>Murdoch Childrens Research Institute, Melbourne, VIC, Australia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Department of Paediatrics, University of Melbourne, Melbourne, VIC, Australia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Department of Allergy and Immunology, Royal Children's Hospital, Melbourne, VIC, Australia.</Affiliation></AffiliationInfo></Author></AuthorList><Language>eng</Language><GrantList CompleteYN="Y"><Grant><GrantID>R18 AI048693</GrantID><Acronym>AI</Acronym><Agency>NIAID NIH HHS</Agency><Country>United States</Country></Grant></GrantList><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D016454">Review</PublicationType></PublicationTypeList><ArticleDate DateType="Electronic"><Year>2015</Year><Month>10</Month><Day>07</Day></ArticleDate></Article><MedlineJournalInfo><Country>Netherlands</Country><MedlineTA>J Clin Immunol</MedlineTA><NlmUniqueID>8102137</NlmUniqueID><ISSNLinking>0271-9142</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><CommentsCorrectionsList><CommentsCorrections RefType="Cites"><RefSource>Lupus. 2013 May;22(6):639-43</RefSource><PMID Version="1">23651859</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>Immunol Res. 2014 Oct;60(1):132-44</RefSource><PMID Version="1">24668296</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>Front Immunol. 2013 Oct 31;4:351</RefSource><PMID Version="1">24198817</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>Ann Allergy Asthma Immunol. 2014 Jun;112(6):489-95</RefSource><PMID Version="1">24860921</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>J Clin Immunol. 2013 Aug;33(6):1078-87</RefSource><PMID Version="1">23657403</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>Inflamm Bowel Dis. 2012 Nov;18(11):2120-7</RefSource><PMID Version="1">22434667</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>Nucleic Acids Res. 2015 Jan;43(Database issue):D789-98</RefSource><PMID Version="1">25428349</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>Science. 2015 Apr 24;348(6233):448-53</RefSource><PMID Version="1">25814066</PMID></CommentsCorrections><CommentsCorrections RefType="Cites"><RefSource>J Clin Immunol. 2013 Jan;33(1):1-7</RefSource><PMID Version="1">22847546</PMID></CommentsCorrections></CommentsCorrectionsList><MeshHeadingList><MeshHeading><DescriptorName UI="D015551" MajorTopicYN="N">Autoimmunity</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D005104" MajorTopicYN="N">Expert Testimony</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006967" MajorTopicYN="N">Hypersensitivity</DescriptorName><QualifierName UI="Q000276" MajorTopicYN="Y">immunology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D007109" MajorTopicYN="N">Immunity</DescriptorName><QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D007153" MajorTopicYN="N">Immunologic Deficiency Syndromes</DescriptorName><QualifierName UI="Q000145" MajorTopicYN="N">classification</QualifierName><QualifierName UI="Q000276" MajorTopicYN="Y">immunology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D007239" MajorTopicYN="N">Infection</DescriptorName><QualifierName UI="Q000276" MajorTopicYN="Y">immunology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D007249" MajorTopicYN="N">Inflammation</DescriptorName><QualifierName UI="Q000276" MajorTopicYN="Y">immunology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D009369" MajorTopicYN="N">Neoplasms</DescriptorName><QualifierName UI="Q000276" MajorTopicYN="Y">immunology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D010641" MajorTopicYN="N">Phenotype</DescriptorName></MeshHeading></MeshHeadingList><OtherID Source="NLM">PMC4659854</OtherID><KeywordList Owner="NOTNLM"><Keyword MajorTopicYN="N">IUIS PID expert committee</Keyword><Keyword MajorTopicYN="N">Primary immunodeficiencies</Keyword><Keyword MajorTopicYN="N">classification</Keyword></KeywordList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="received"><Year>2015</Year><Month>08</Month><Day>11</Day></PubMedPubDate><PubMedPubDate PubStatus="accepted"><Year>2015</Year><Month>09</Month><Day>16</Day></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2015</Year><Month>10</Month><Day>9</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2015</Year><Month>10</Month><Day>9</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2016</Year><Month>9</Month><Day>7</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">26445875</ArticleId><ArticleId IdType="doi">10.1007/s10875-015-0198-5</ArticleId><ArticleId IdType="pii">10.1007/s10875-015-0198-5</ArticleId><ArticleId IdType="pmc">PMC4659854</ArticleId></ArticleIdList></PubmedData></pubmed><affiliations><list><country><li>Allemagne</li><li>Australie</li><li>Colombie</li><li>France</li><li>Israël</li><li>Japon</li><li>Koweït</li><li>Maroc</li><li>Royaume-Uni</li><li>États-Unis</li></country><region><li>Angleterre</li><li>Bavière</li><li>Californie</li><li>District de Haute-Bavière</li><li>Grand Londres</li><li>Maryland</li><li>Massachusetts</li><li>Pennsylvanie</li><li>Washington (État)</li><li>État de New York</li><li>Île-de-France</li></region><settlement><li>Londres</li><li>Munich</li><li>Paris</li></settlement></list><tree><country name="Maroc"><noRegion><name sortKey="Bousfiha, Aziz" sort="Bousfiha, Aziz" uniqKey="Bousfiha A" first="Aziz" last="Bousfiha">Aziz Bousfiha</name></noRegion><name sortKey="Ailal, Fatima" sort="Ailal, Fatima" uniqKey="Ailal F" first="Fatima" last="Ailal">Fatima Ailal</name><name sortKey="Jeddane, Leila" sort="Jeddane, Leila" uniqKey="Jeddane L" first="Leïla" last="Jeddane">Leïla Jeddane</name></country><country name="Koweït"><noRegion><name sortKey="Al Herz, Waleed" sort="Al Herz, Waleed" uniqKey="Al Herz W" first="Waleed" last="Al-Herz">Waleed Al-Herz</name></noRegion></country><country name="États-Unis"><region name="État de New York"><name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name></region><name sortKey="Chatila, Talal" sort="Chatila, Talal" uniqKey="Chatila T" first="Talal" last="Chatila">Talal Chatila</name><name sortKey="Conley, Mary Ellen" sort="Conley, Mary Ellen" uniqKey="Conley M" first="Mary Ellen" last="Conley">Mary Ellen Conley</name><name sortKey="Cunningham Rundles, Charlotte" sort="Cunningham Rundles, Charlotte" uniqKey="Cunningham Rundles C" first="Charlotte" last="Cunningham-Rundles">Charlotte Cunningham-Rundles</name><name sortKey="Holland, Steven M" sort="Holland, Steven M" uniqKey="Holland S" first="Steven M" last="Holland">Steven M. Holland</name><name sortKey="Ochs, Hans D" sort="Ochs, Hans D" uniqKey="Ochs H" first="Hans D" last="Ochs">Hans D. Ochs</name><name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name><name sortKey="Puck, Jennifer M" sort="Puck, Jennifer M" uniqKey="Puck J" first="Jennifer M" last="Puck">Jennifer M. Puck</name><name sortKey="Sullivan, Kathleen E" sort="Sullivan, Kathleen E" uniqKey="Sullivan K" first="Kathleen E" last="Sullivan">Kathleen E. Sullivan</name></country><country name="Israël"><noRegion><name sortKey="Etzioni, Amos" sort="Etzioni, Amos" uniqKey="Etzioni A" first="Amos" last="Etzioni">Amos Etzioni</name></noRegion></country><country name="Colombie"><noRegion><name sortKey="Franco, Jose Luis" sort="Franco, Jose Luis" uniqKey="Franco J" first="Jose Luis" last="Franco">Jose Luis Franco</name></noRegion></country><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Gaspar, H Bobby" sort="Gaspar, H Bobby" uniqKey="Gaspar H" first="H Bobby" last="Gaspar">H Bobby Gaspar</name></region></country><country name="Allemagne"><region name="Bavière"><name sortKey="Klein, Christoph" sort="Klein, Christoph" uniqKey="Klein C" first="Christoph" last="Klein">Christoph Klein</name></region></country><country name="Japon"><noRegion><name sortKey="Nonoyama, Shigeaki" sort="Nonoyama, Shigeaki" uniqKey="Nonoyama S" first="Shigeaki" last="Nonoyama">Shigeaki Nonoyama</name></noRegion></country><country name="France"><region name="Île-de-France"><name sortKey="Oksenhendler, Eric" sort="Oksenhendler, Eric" uniqKey="Oksenhendler E" first="Eric" last="Oksenhendler">Eric Oksenhendler</name></region></country><country name="Australie"><noRegion><name sortKey="Tang, Mimi L K" sort="Tang, Mimi L K" uniqKey="Tang M" first="Mimi L K" last="Tang">Mimi L K. 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