Primary immunodeficiency diseases: An update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005

Although relatively rare, primary immunodeficiency diseases (PIDs) provide an excellent window into the functioning of the immune system. In the late 1960s, observations on these diseases, with their associated infections and genetics, bisected the immune system into humoral immunity and cell-mediat...

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Bibliographic Details
Published in:Journal of allergy and clinical immunology 2006-04, Vol.117 (4), p.883-896
Main Authors: Notarangelo, Luigi, Casanova, Jean-Laurent, Conley, Mary Ellen, Chapel, Helen, Fischer, Alain, Puck, Jennifer, Roifman, Chaim, Seger, Reinhard, Geha, Raif S.
Format: Article
Language:English
Subjects:
B cells
B-Lymphocytes - immunology
Classification
complement
Complement System Proteins - deficiency
Defects
Disease
Genes
Humans
Hungary
immune dysregulation syndromes
Immune system
Immunity, Innate
Immunologic Deficiency Syndromes - classification
Immunologic Deficiency Syndromes - diagnosis
Immunologic Deficiency Syndromes - genetics
Immunologic Deficiency Syndromes - immunology
Infections
innate immunity
Ligands
Mutation
phagocytes
Phagocytes - immunology
Primary immunodeficiency diseases
T cells
T-Lymphocytes - immunology
Terminology as Topic
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Summary:Although relatively rare, primary immunodeficiency diseases (PIDs) provide an excellent window into the functioning of the immune system. In the late 1960s, observations on these diseases, with their associated infections and genetics, bisected the immune system into humoral immunity and cell-mediated immunity. These diseases also represent a challenge in their diagnosis and treatment. Beginning in 1970, a unified nomenclature for the then-known PIDs was created by a committee convoked by the World Health Organization. Since then, and later under the aegis of the International Union of Immunological Societies, an international committee of experts has met every 2 to 3 years to update the classification of PIDs. During the past 15 years, the molecular basis of more than 120 PIDs has been elucidated. This update results from the latest meeting of this committee in Budapest, Hungary, in June 2005, which followed 2½ days of scientific discussions. As a result of this work, new entities have been included, and the nomenclature of some PIDs (specifically of the various forms of class-switch recombination defects, previously known as hyper-IgM syndromes) has been changed.
ISSN:0091-6749
1097-6825
DOI:10.1016/j.jaci.2005.12.1347