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. 2013 Feb;171(2):186-94.
doi: 10.1111/cei.12002.

Efficiency of immunoglobulin G replacement therapy in common variable immunodeficiency: correlations with clinical phenotype and polymorphism of the neonatal Fc receptor

V Gouilleux-Gruart  1 , H Chapel, S Chevret, M Lucas, M Malphettes, C Fieschi, S Patel, D Boutboul, M-N Marson, L Gérard, M Lee, H Watier, E Oksenhendler; DEFI study group
Collaborators, Affiliations

Efficiency of immunoglobulin G replacement therapy in common variable immunodeficiency: correlations with clinical phenotype and polymorphism of the neonatal Fc receptor

V Gouilleux-Gruart et al. Clin Exp Immunol. 2013 Feb.

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.

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Figures

Fig. 1
Fig. 1
(a) Immunoglobulin (Ig)G efficiency index in common variable immunodeficiency (CVID) patients according to the route of replacement therapy or the presence of a disease-related complication (i.e. lymphoid hyperplasia, autoimmune cytopenia or enteropathy). (b) The most significant difference in efficiency was observed according to the route of substitution in patients belonging to the ‘infections only’ group. Data are represented as box-plots displaying medians, 25th and 75th percentiles as boxes, and 10th and 90th percentiles as whiskers. Difference between groups were compared by the Kruskal–Wallis test.
Fig. 2
Fig. 2
(a) Immunoglobulin (Ig)G efficiency index in common variable immunodeficiency (CVID) patients treated with intravenous immunoglobulin (IVIG) according to the presence of a disease-related complication (i.e. lymphoid hyperplasia, autoimmune cytopenia or enteropathy) and to the genotype of the Fc fragment of IgG, receptor, transporter, alpha (FCGRT) variable number of tandem repeats (VNTR) promoter gene (VNTR3/3 versus VNTRnon-3/3). (b) A significant difference in efficiency was observed according to the FCGRT_VNTR genotype in patients presenting with a disease-related complication of CVID. Data are represented as box plots displaying medians, 25th and 75th percentiles as boxes, and 10th and 90th percentiles as whiskers. Difference between groups were compared by the Kruskal-Wallis test.
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