title: Efficiency of immunoglobulin G replacement therapy in common variable immunodeficiency: correlations with clinical phenotype and polymorphism of the neonatal Fc receptor.
abstract: Treatment of common variable immunodeficiency disorders (CVID) is based on replacement therapy using intravenous (i.v.) or subcutaneous (s.c.) immunoglobulin (Ig)G. Interindividual variation of IgG dose is common. A total of 380 CVID patients on stable IgG replacement from two prospective cohorts were analysed. An 'efficiency' index was defined as the ratio of serum IgG trough level minus IgG residual to the average weekly dose of IgG infusion. A reduced efficiency of IgG was associated independently with the i.v. route (P < 0·001) and with the presence of at least one CVID disease-related phenotype (lymphoproliferation, autoimmune cytopenia or enteropathy) (P < 0·001). High IgG efficiency was noted in patients homozygotes for the variable number tandem repeat (VNTR) 3/3 polymorphism of the neonatal Fc receptor gene [IgG Fc fragment receptor transporter alpha chain (FCGRT)] promoter, and this was particularly significant in patients treated with IVIG (P < 0.01). In a multivariate analysis, FCGRT VNTR 3/3 genotype (P = 0·008) and high serum albumin (P < 0·001) were associated independently with increased efficiency of i.v. Ig.
authors.affiliation: CNRS UMR 7292, Université François Rabelais, CHRU de Tours, Tours, France.
authors.forename: V
authors.initials: V
authors.lastname: Gouilleux-Gruart
authors.forename: H
authors.initials: H
authors.lastname: Chapel
authors.forename: S
authors.initials: S
authors.lastname: Chevret
authors.forename: M
authors.initials: M
authors.lastname: Lucas
authors.forename: M
authors.initials: M
authors.lastname: Malphettes
authors.forename: C
authors.initials: C
authors.lastname: Fieschi
authors.forename: S
authors.initials: S
authors.lastname: Patel
authors.forename: D
authors.initials: D
authors.lastname: Boutboul
authors.forename: M-N
authors.initials: MN
authors.lastname: Marson
authors.forename: L
authors.initials: L
authors.lastname: Gérard
authors.forename: M
authors.initials: M
authors.lastname: Lee
authors.forename: H
authors.initials: H
authors.lastname: Watier
authors.forename: E
authors.initials: E
authors.lastname: Oksenhendler
mesh_terms: D000328:Adult; D054316:Biomarkers, Pharmacological; D015331:Cohort Studies; D017074:Common Variable Immunodeficiency / Q000188:drug therapy* / Q000276:immunology; D005260:Female; D015395:Histocompatibility Antigens Class I / Q000235:genetics*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage* / Q000009:adverse effects; D007279:Injections, Subcutaneous; D008297:Male; D008875:Middle Aged; D018598:Minisatellite Repeats / Q000235:genetics; D010641:Phenotype; D011110:Polymorphism, Genetic; D011401:Promoter Regions, Genetic / Q000235:genetics; D011446:Prospective Studies; D011961:Receptors, Fc / Q000235:genetics*; D015533:Transcriptional Activation / Q000235:genetics; D016896:Treatment Outcome
chemical_list: D054316:Biomarkers, Pharmacological; D015395:Histocompatibility Antigens Class I; D016756:Immunoglobulins, Intravenous; D011961:Receptors, Fc; C100688:Fc receptor, neonatal