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Inicio Allergologia et Immunopathologia The profile of IL-4, IL-5, IL-10 and GATA3 in patients with LRBA deficiency and ...
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Vol. 47. Issue 2.
Pages 172-178 (March - April 2019)
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Vol. 47. Issue 2.
Pages 172-178 (March - April 2019)
Original Article
DOI: 10.1016/j.aller.2018.06.003
The profile of IL-4, IL-5, IL-10 and GATA3 in patients with LRBA deficiency and CVID with no known monogenic disease: Association with disease severity
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G. Azizia,b,c,d, Y. Bagherie,f, R. Yazdanic, M. Zaki-Dizajig, M. Jameeh, F. Jadidi-Niaraghi,j, A.N. Kamalik, H. Abolhassanic,l, A. Aghamohammadic,
Corresponding author
aghamohammadi@tums.ac.ir

Corresponding author.
a Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
b Department of Immunology, School of Medicine, Alborz University of Medical Sciences, Karaj, Iran
c Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
d Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
e Clinical Research Development Unit (CRDU), 5 Azar Hospital, Golestan University of Medical Sciences, Gorgan, Iran
f Department of Allergy and Clinical Immunology, Iran University of Medical Sciences, Tehran, Iran
g Department of Medical Genetics, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
h Student Research Committee, Alborz University of Medical Sciences, Alborz, Iran
i Immunology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
j Department of Immunology, School of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran
k CinnaGen Medical Biotechnology Research Center, Alborz University of Medical Sciences, Karaj, Iran
l Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden
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Table 1. Demographic and corresponding immunologic and clinical data for patients and control.
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Abstract
Background

Common variable immunodeficiency (CVID) is the most common symptomatic form of primary immunodeficiency (PID). LPS-responsive beige-like anchor protein (LRBA) deficiency is an autosomal recessive disease characterized by a CVID-like phenotype. T cell abnormality was reported in patients with CVID and LRBA deficiency. The study's aim was to evaluate IL-4, IL-5, IL-10 and GATA3 expression in patients with LRBA deficiency and CVID with no known monogenic disease, and further evaluate its relevance with immunological futures and clinical complications of patients.

Methods

The study population comprised patients with CVID, LRBA deficiency and age–sex matched healthy controls. Mutation analysis was done by whole exome sequencing in CVID patients to rule out monogenic PIDs. After CD4+ T cell stimulation with anti-CD3 and anti-CD28 monoclonal antibodies, gene expression of IL-4, IL-5, IL-10 and transcription factor GATA3 was evaluated by real-time polymerase chain reaction. The protein of mentioned cytokines was assessed by enzyme-linked immunosorbent assay.

Results

The main clinical presentations of CVID patients were infections only and lymphoproliferations phenotypes, but in LRBA patients were autoimmune and enteropathy phenotype. The frequencies of CD4+ T cells were significantly reduced in LRBA and CVID patients. There were no statistically significant differences among GATA3, IL4, and IL5 gene expressions by CD4+ T cells of patients and controls, however, the IL10 expressions in CVID patients was significantly lower than in LRBA patients and HCs. As compared with HCs, CVID patients showed a prominent decrease in IL-4 and IL-10 production by CD4+ T cells.

Conclusions

Our findings demonstrated that patients with CVID and LRBA deficiency (even with severe infectious and inflammatory complications) have not imbalance in Th2 response, which is in parallel with lower frequency of allergy and asthma in these patients.

Keywords:
Common variable immunodeficiency
LRBA deficiency
T-helper 2 cell
IL-4
IL-5
IL-10

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