Low percentages of regulatory T cells in common variable immunodeficiency (CVID) patients with autoimmune diseases and its association with increased numbers of CD4+CD45RO+ T and CD21low B cells

Immune Monitoring of Patients With Primary Immune Regulation Disorders Unravels Higher Frequencies of Follicular T Cells With Different Profiles That Associate With Alterations in B Cell Subsets

“…It has been proposed that they develop from MBL that have undergone chronic stimulation associated with cTfh1 and IFN-g dysregulation (79)(80)(81). However, in our study, we observed increased frequencies of CD21 low B cells only in CVID dys patients that also exhibited increased frequencies of TBL and reduced frequencies of Sw-MBL as described by others (82)(83)(84)(85). Therefore, the concomitant accumulation of CD21 low B cells and immature/naïve cells in detriment of activated/effector cells that we observed in these patients, together with additional observations that indicate that CD21 low B cells are IgM + IgD + CD27support the idea that CD21 low B cells exhibit phenotypic features more closely related to naïve B cells or B cells in early stages of activation.…”

Functions of Tfh Cells in Common Variable Immunodeficiency

“…Numerous studies have reported abnormalities in the T-cell compartment [as reviewed in (27)], which is not surprising given the central role of T cells, especially CD4 T cells, in B cell activation and differentiation into memory and Ig-producing cells. Patients with complications usually have low numbers of naive CD4 T cells but increased activated CD4 T cell counts (28)(29)(30), defective T cell functions (lower proliferative capacities, abnormalities in cytokine production) and reduced levels of regulatory T cells (31). Given their function as B helper cells, TFH represent a CD4 T cell subset of great interest in CVID pathogenesis and will now be discussed.…”

Enfermedades autoimunes en pacientes con inmunodeficiencia común variable

“…49 Varios grupos han encontrado defectos en las células Treg, tanto en número como en función, en pacientes con IDCV, particularmente en aquellos con autoinmunidad. 50,51 Se han identificado dos defectos moleculares que afectan la función de las células Treg: la haploinsuficiencia de CTLA-4, un receptor que actúa como regulador negativo en las células Treg y la deficiencia de CTLA-4, que deteriora la función de estas células y lleva a procesos de autoinmunidad sistémica severa con progresiva hipogammaglobulinemia. 52 La deficiencia de la proteína LRBA causa un síndrome similar a IDCV con autoinmunidad.…”

“…76 Rev Alerg Mex. 2021;68(1): [48][49][50][51][52][53][54][55][56][57][58][59][60][61][62][63][64] Berrón-Ruiz L. Autoinmunidad en IDCV Aún faltan ensayos prospectivos sobre la efectividad de los fármacos inmunosupresores en la IDCV. La esperanza de vida de los pacientes con IDCV ha mejorado considerablemente durante los últimos 30 años, desde los primeros 12 años hasta más de 50 años en la actualidad.…”