Autoimmunity and its association with regulatory T cells and B cell subsets in patients with common variable immunodeficiency
Allergol. immunopatol
; 46(2): 127-135, mar.-abr. 2018. tab, graf
Artigo
em Inglês
| IBECS
| ID: ibc-172170
Biblioteca responsável:
ES1.1
Localização: BNCS
ABSTRACT
Background:
Common variable immunodeficiency (CVID) is one of the most prevalent symptomatic primary immunodeficiencies (PIDs), which manifests a wide clinical variability such as autoimmunity, as well as T cell and B cell abnormalities.Methods:
A total of 72 patients with CVID were enrolled in this study. Patients were evaluated for clinical manifestations and classified according to the presence or absence of autoimmune disease. We measured regulatory T cells (Tregs) and B-cell subsets using flow cytometry, as well as specific antibody response (SAR) to pneumococcal vaccine, autoantibodies and anti-IgA in patients.Results:
Twenty-nine patients (40.3%) have shown at least one autoimmune manifestation. Autoimmune cytopenias and autoimmune gastrointestinal diseases were the most common. A significant association was detected between autoimmunity and presence of hepatomegaly and splenomegaly. Among CVID patients, 38.5% and 79.3% presented a defect in Tregs and switched memory B-cells, respectively, whereas 69.0% presented CD21low B cell expansion. Among patients with a defect in Treg, switched memory and CD21low B cell, the frequency of autoimmunity was 80.0%, 52.2% and 55.0%, respectively. A negative correlation was observed between the frequency of Tregs and CD21low B cell population. 82.2% of patients had a defective SAR which was associated with the lack of autoantibodies.Conclusions:
Autoimmunity may be the first clinical manifestation of CVID, thus routine screening of immunoglobulins is suggested for patients with autoimmunity. Lack of SAR in CVID is associated with the lack of specific autoantibodies in patients with autoimmunity. It is suggested that physicians use alternative diagnostic procedures (AU)RESUMEN
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Bases de dados nacionais
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Espanha
Base de dados:
IBECS
Assunto principal:
Doenças Autoimunes
/
Subpopulações de Linfócitos B
/
Imunodeficiência de Variável Comum
/
Linfócitos T Reguladores
Tipo de estudo:
Fatores de risco
Limite:
Humanos
Idioma:
Inglês
Revista:
Allergol. immunopatol
Ano de publicação:
2018
Tipo de documento:
Artigo
Instituição/País de afiliação:
Alborz University of Medical Sciences/Iran
/
Shahid Beheshti University of Medical Sciences/Iran
/
Tehran University of Medical Sciences/Iran
/
University of Tehran/Iran