| Tolerance and autoimmunity: lessons at the bedside of primary immunodeficiencies. | |
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MedLine Citation:
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PMID: 17869610 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The recent progress in the genetic characterization of many primary immunodeficiencies (PIDs) allows for a better understanding of immune molecular and cellular mechanisms. The present chapter discusses associations between PIDs and autoimmune diseases (AIDs) in this new light. PIDs are classified according to the frequency of association with AIDs, defining four groups of conditions: systematic (more than 80% of all patients), strong (10-80%), mild (less than 10%), and absent (no available descriptions). Several general conclusions could be drawn: (1) pathological autoimmune (AI) manifestations are very frequently associated with PIDs, indicating that, contrary to conventional notions, antimicrobial protection and natural tolerance to body tissues share many basic mechanisms; (2) in some gene defects, association is so strong that one could speak of "monogenic" AIDs; (3) basic types of PIDs are selectively associated with AID of a particular set of target tissues; (4) while for some gene defects, current theory satisfactorily explains pathogenesis of the corresponding AID, other situations suggest extensive gaps in the present understanding of natural tolerance; and (5) not exceptionally, observations on the AI phenotype for the same gene defect in mouse and man are not concordant, perhaps owing to the limited genetic diversity of mouse models, often limited to a single mouse strain. Overall, clinical observations on PID support the new paradigm of "dominant" tolerance to self-components, in which AID owes to deficits in immune responses (i.e., in regulatory mechanisms), rather than from excessive reactivity. |
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Authors:
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Magda Carneiro-Sampaio; Antonio Coutinho |
Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: Advances in immunology Volume: 95 ISSN: 0065-2776 ISO Abbreviation: Adv. Immunol. Publication Date: 2007 |
Date Detail:
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Created Date: 2007-09-17 Completed Date: 2007-12-06 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0370425 Medline TA: Adv Immunol Country: United States |
Other Details:
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Languages: eng Pagination: 51-82 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, Children's Hospital, Faculdade de Medicina da Universidade de São Paulo, Brazil. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Autoimmune Diseases / genetics, immunology*, metabolism Autoimmunity* B-Lymphocytes / immunology Humans Immune Tolerance* Immunologic Deficiency Syndromes / immunology* T-Lymphocytes / immunology Thymus Gland / immunology |
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