Document Detail


Common variable immunodeficiency: etiological and treatment issues.
MedLine Citation:
PMID:  19571563     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
One of the great advances in clinical medicine was the recognition of the pleomorphism of the immune response and the multiple afferent and efferent limbs of antigen processing and responsiveness. A significant contribution to this understanding was derived from studies of human immunodeficiency states, including both inherited and acquired syndromes. Amongst these syndromes, one of the most common, and least understood, is common variable immune deficiency (CVID). CVID is a syndrome that leads to a reduction in serum immunoglobulins and complications including recurrent infections. Management includes immunoglobulin replacement therapy; however, patients with CVID are at risk for complications of exogenous immunoglobulin administration as well as CVID-associated diseases such as autoimmune processes and malignancies. To assess the current state of knowledge in the field, we performed a literature review of a total of 753 publications covering the period of 1968 until 2008. From this list, 189 publications were selected for discussion. In this review, we demonstrate that while the molecular basis of CVID in many cases remains incompletely understood, significant strides have been made and it is now clear that there is involvement of several pathways of immune activation, with contributions from both T and B cells. Furthermore, despite the current gaps in our knowledge of the molecular pathogenesis of the syndrome, there have been dramatic advances in management that have led to improved survival and significantly reduced morbidity in affected patients.
Authors:
Sean Deane; Carlo Selmi; Stanley M Naguwa; Suzanne S Teuber; M Eric Gershwin
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review     Date:  2009-07-01
Journal Detail:
Title:  International archives of allergy and immunology     Volume:  150     ISSN:  1423-0097     ISO Abbreviation:  Int. Arch. Allergy Immunol.     Publication Date:  2009  
Date Detail:
Created Date:  2009-11-20     Completed Date:  2010-01-11     Revised Date:  2010-11-02    
Medline Journal Info:
Nlm Unique ID:  9211652     Medline TA:  Int Arch Allergy Immunol     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  311-24     Citation Subset:  IM    
Copyright Information:
Copyright 2009 S. Karger AG, Basel.
Affiliation:
Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, California 95616, USA.
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MeSH Terms
Descriptor/Qualifier:
Animals
Anti-Bacterial Agents / therapeutic use*
Autoimmune Diseases / etiology,  prevention & control
B-Lymphocytes / immunology*
Common Variable Immunodeficiency / complications,  genetics,  immunology*,  therapy*
Humans
Immunoglobulins, Intravenous / therapeutic use*
Infection / etiology,  therapy
Lymphocyte Activation / genetics,  immunology
Monitoring, Physiologic
Neoplasms / etiology,  prevention & control
T-Lymphocytes / immunology
Chemical
Reg. No./Substance:
0/Anti-Bacterial Agents; 0/Immunoglobulins, Intravenous
Comments/Corrections
Erratum In:
Int Arch Allergy Immunol. 2010;151(4):284
Note: Dosage error in article text

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