Common variable immunodeficiency: the immune system in chaos.
Common variable immunodeficiency (CVID) is a heterogeneous disorder that is associated with low serum-immunoglobulin concentrations, defective specific-antibody production and an increased susceptibility to bacterial infections of the respiratory and gastrointestinal tracts. In spite of the identification of genes that are associated with several known primary immunodeficiencies, the basic immunologic and molecular defects of the majority of patients with CVID have remained obscure. Most of the studies aimed at understanding the immunopathogenesis of CVID suggest that this condition is primarily a T-cell disorder, although renewed attention on the genetic linkage and haplotype analysis in families of patients with CVID and on the role of dendritic cells and B cells has revealed several interesting features. This new information should assist in understanding the pathogenesis of CVID and improving the therapeutic strategies.
INSERM Unité 681 and Université Pierre et Marie Curie, Institut des Cordeliers, 15 rue de l'Ecole de Médecine, Paris 75006, France.
Jagadeesh
J
Bayry
Olivier
O
Hermine
David A
DA
Webster
Yves
Y
Lévy
Srini V
SV
Kaveri
D001402:B-Lymphocytes / Q000502:physiology; D017074:Common Variable Immunodeficiency / Q000235:genetics* / Q000276:immunology*; D003713:Dendritic Cells / Q000502:physiology; D008040:Genetic Linkage; D006239:Haplotypes; D006801:Humans; D013601:T-Lymphocytes / Q000502:physiology