Decrease in phenotypic regulatory T cells in subsets of patients with common variable immunodeficiency

Horn, Julia; Manguiat, Annabelle; Berglund, Lucinda J.; Knerr, Viviane; Tahami, Fariba; Grimbacher, Bodo; Fulcher, David A.

doi:10.1111/j.1365-2249.2009.03913.x

Cited by 68 publications

(44 citation statements)

References 27 publications

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“…There is a significant correlation between low numbers of Treg cells and autoimmune manifestations [61,63,65,66], granulomatous lesions [65], and splenomegaly [61]. Genre et al [66] reported that CVID patients who were likely to develop autoimmunity had a significantly reduced frequency of Treg cells accompanied by decreased intensity of Foxp3 expression.…”

Section: Regulatory T-cell Defectsmentioning

confidence: 99%

T-Cell Abnormalities in Common Variable Immunodeficiency

Azizi

Rezaei

Kiaee

et al. 2016

J Investig Allergol Clin Immunol

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Common variable immunodeficiency (CVID) is the most common clinical primary immunodeficiency. It is characterized by a defect in B-cell differentiation to plasma and memory B cells. Moreover, numerous T-cell abnormalities have been reported and include decreased T-cell count and proliferative response, increased T-cell activation and apoptosis, and abnormalities in cytokine production. The aims of this review are to describe phenotypic and functional defects in T cells in CVID patients and to review the literature with respect to the effects of immunoglobulin replacement on the T-cell component in CVID patients. Key words: Common variable immunodeficiency. T cell. Helper T cells. Regulatory T cells. ResumenLa inmunodeficiencia común variable (CVID) es la inmunodeficiencia primaria más frecuente. Se caracteriza por un defecto en la diferenciación de linfocitos B hacia células plasmáticas y linfocitos B memoria. Se han descrito numerosas alteraciones en los linfocitos T de estos pacientes, tales como disminución en el número de linfocitos T y en sus respuestas proliferativas, aumento en la activación de células T y en la apoptosis, así como alteraciones en la producción de citokinas. El objetivo de esta revisión es describir las alteraciones funcionales y fenotípicas de los linfocitos T en los pacientes con CVID y revisar la bibliografía en relación a los efectos que la administración de inmunoglobulinas produce en los linfocitos T de los pacientes con CVID. Palabras clave: Inmunodeficiencia común variable. Células T. Células T helper. Células T reguladoras.

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Section: Regulatory T-cell Defectsmentioning

confidence: 99%

T-Cell Abnormalities in Common Variable Immunodeficiency

Azizi

Rezaei

Kiaee

et al. 2016

J Investig Allergol Clin Immunol

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“…Clinically, a severe reduction in SMB is associated with a higher risk of granulomatous disease and splenomegaly (39), suggesting that a most profound lack of B cell maturation is likely to be associated with the abnormal cellular or cytokine environment that supports granuloma formation (20). CVID patients had significantly fewer Treg than controls, and a low frequency of Treg is clinically associated with splenomegaly (46,47), granulomatous disease (48), and autoimmune cytopenia (49). The suppressive function of Treg from CVID subjects with autoimmune disease is significantly attenuated compared to CVID subjects with no autoimmune disease and to healthy controls, and the expression of several proteins associated with Treg function (FoxP3, Granzyme A, XCL1, pSTAT5, and GITR) is decreased in the same patients (50).…”

Section: The Reduction Of Cd27+igd-igm-switched Memory B Cells (Smb) mentioning

confidence: 99%

Common variable immunodeficiency: Crossroads between infections, inflammation and autoimmunity

Baldovino

Montin

Martino

et al. 2013

Autoimmunity Reviews

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“…B cell receptor (BCR) and TLR7 and TLR9 responses are found altered in most CVID patients (3)(4)(5). However, CVID patients often have additional immunological abnormalities not apparently attributable to B cells, such as Treg deficiency, a finding associated with clinical autoimmunity (6,7). Although the majority of CVID cases have no identifiable pathogenic mutation, 7%-10% of patients bear a mutation in the TNFRSF13B gene encoding TACI, a tumor necrosis factor receptor superfamily member expressed on B cells (8,9).…”

Section: Introductionmentioning

confidence: 99%

CVID-associated TACI mutations affect autoreactive B cell selection and activation

Romberg¹,

Chamberlain²,

Saadoun³

et al. 2013

J. Clin. Invest.

152

198

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Common variable immune deficiency (CVID) is an assorted group of primary diseases that clinically manifest with antibody deficiency, infection susceptibility, and autoimmunity. Heterozygous mutations in the gene encoding the tumor necrosis factor receptor superfamily member TACI are associated with CVID and autoimmune manifestations, whereas two mutated alleles prevent autoimmunity. To assess how the number of TACI mutations affects B cell activation and tolerance checkpoints, we analyzed healthy individuals and CVID patients carrying one or two TACI mutations. We found that TACI interacts with the cleaved, mature forms of TLR7 and TLR9 and plays an important role during B cell activation and the central removal of autoreactive B cells in healthy donors and CVID patients. However, only subjects with a single TACI mutation displayed a breached immune tolerance and secreted antinuclear antibodies (ANAs). These antibodies were associated with the presence of circulating B cell lymphoma 6-expressing T follicular helper (Tfh) cells, likely stimulating autoreactive B cells. Thus, TACI mutations may favor CVID by altering B cell activation with coincident impairment of central B cell tolerance, whereas residual B cell responsiveness in patients with one, but not two, TACI mutations enables autoimmune complications.

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Decrease in phenotypic regulatory T cells in subsets of patients with common variable immunodeficiency

Cited by 68 publications

References 27 publications

T-Cell Abnormalities in Common Variable Immunodeficiency

T-Cell Abnormalities in Common Variable Immunodeficiency

Common variable immunodeficiency: Crossroads between infections, inflammation and autoimmunity

CVID-associated TACI mutations affect autoreactive B cell selection and activation

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