Role of B cells in common variable immune deficiency

Ahn, Sam S.; Cunningham‐Rundles, Charlotte

doi:10.1586/eci.09.43

Cited by 58 publications

(43 citation statements)

References 80 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…CVID is the most common symptomatic immune disorder in adults with a deficiency of switched memory B cells (5,6). Although adequate Ig replacement in addition to use of prophylactic antibiotics and monoclonal antibodies such as infliximab, rituximab, adalimumab etc.…”

Section: Discussionmentioning

confidence: 99%

“…Although IgRT, use of prophylactic antibiotics and monoclonal antibodies such as infliximab, rituximab, adalimumab etc. reduce the frequency of infections, complications due to recurrent and frequent infections of the respiratory tract, particularly bronchiectasis and granulomatous lung disease, are still challenging and occur in approximately 20% of patients (5). In this study, we aimed to investigate the risk factors associated with bronchiectasis in adult patients with CVID who were followed-up at our Clinical Immunology Department.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Risk Factors of Bronchiectasis in Adult Patients with Common Variable Immunodeficiency

Aytekin¹,

Çölkesen²,

Yıldız³

et al. 2019

Asthma Allergy Immunol

View full text Add to dashboard Cite

Objective: Common Variable Immunodeficiency (CVID) is a heterogeneous immune disorder characterized by frequent and recurrent upper and lower respiratory tract infections. Although immunoglobulin (Ig) replacement therapy (IgRT) reduces the frequency of infections, complications due to infections of the respiratory tract, particularly bronchiectasis, is still challenging and occur in approximately 20% of patients. Therefore, we aimed to identify independent predictors of bronchiectasis in adult patients with CVID. Materials and Methods:We retrospectively analyzed the hospital records of 45 CVID patients (26 male, 19 female). Additionally, we classified the patients according to the presence of bronchiectasis. Bronciectasis was confirmed by high resolution computed tomography of thorax.Results: Forty-five patients with CVID (F:19 [42.2%], M: 26 [57.8%]) were included in the study (age: 36.52 ± 12.86). Bronchiectasis was observed in 60% of the study group. Univariate regression analysis revealed that IgG levels (OR: 4.889, 95% CI, 0.485-0.959, p: 0.012) and IgA levels (OR: 2.559, 95% CI, 0.097-1.235, p: 0.030) at the time of diagnosis and the percentage of switched memory B cells (OR: 0.848, 95% CI, 0.730-0.984, p: 0.030) were significantly associated with bronchiectasis. Multivariate regression analysis showed that a lower percentage of switched memory B cells (OR: 0.853, 95% CI, 0.733-0.991, p: 0.038) and a low platelet count (OR: 1.000, 95% CI, 1.000-1000, p: 0.026) were also independent predictors of bronchiectasis. Conclusion:Clinicians should be alert for CVID patients with reduced switched memory B cells and low platelet counts in order to detect and treat complications associated with bronchiectasis at an early stage.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Risk Factors of Bronchiectasis in Adult Patients with Common Variable Immunodeficiency

Aytekin¹,

Çölkesen²,

Yıldız³

et al. 2019

Asthma Allergy Immunol

View full text Add to dashboard Cite

show abstract

“…Common variable immune deficiency is a complex, heterogeneous disease, with a common feature of ineffective production of high affinity antibodies [33]. The variability in time of the disease onset and clinical symptoms reflects the heterogeneity of defective mechanisms leading to abnormalities in B-cell survival [34], number of circulating CD27 + memory B lymphocytes [7, 8, 13–15, 17, 35], B cell activation after antigen receptor cross-linking [36, 37], T cell signaling [38], and cytokine expression [3, 39].…”

Section: Discussionmentioning

confidence: 99%

Common Variable Immune Deficiency in Children—Clinical Characteristics Varies Depending on Defect in Peripheral B Cell Maturation

et al. 2013

View full text Add to dashboard Cite

Common variable immune deficiency (CVID) is a heterogeneous disease associated with ineffective production of antibodies. It is usually diagnosed in adulthood, but a variable proportion of children develop CVID. Early identification of patients with potentially worse prognosis may help to avoid serious complications. The goal of this study was to associate the clinical phenotype of patients with early onset CVID with peripheral B-cell maturation profile. Four color flow cytometry was used to define distribution of peripheral B-cell subsets in 49 children with early-onset CVID. All clinical data were extracted from medical records. A proportion of patients demonstrated diminishing with time total B-lymphocytes pool, beyond physiological age-related changes. Irrespective from duration of the follow-up period the B-cell maturation profile in individual patients remained unchanged. We identified six different aberrant peripheral B cell maturation profiles associated with different clinical characteristics. Patients with an early B-cell maturation block earlier required replacement therapy and were at significantly greater risk of enteropathy, granuloma formation, cytopenia, and lymphoproliferation. B-cell maturation inhibited at the natural effector stage was associated with higher risk of autoimmune manifestations other than autoimmune cytopenia. Prevalence of male patients was observed among patients with B-cell maturation inhibited at naïve B-cell stage. In conclusion, the diagnostic process in patients with suspected early-onset CVID shall include routine analysis of peripheral B-cell maturation to provide surrogate markers identifying patients at greater risk of developing certain complications.

show abstract

“…It is characterized by defects in differentiation of B cells to plasma cells and memory B cells [1,2]. Affected patients are very susceptible to recurrent infection because of low levels of Ig in serum and to reduced specific antibody responses to protein and polysaccharide antigens [3][4][5][6].…”

Section: Common Variable Immunodeficiency (Cvid) Is the Most Common Cmentioning

confidence: 99%

T-Cell Abnormalities in Common Variable Immunodeficiency

Azizi

Rezaei

Kiaee

et al. 2016

J Investig Allergol Clin Immunol

View full text Add to dashboard Cite

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.

show abstract

Role of B cells in common variable immune deficiency

Cited by 58 publications

References 80 publications

Risk Factors of Bronchiectasis in Adult Patients with Common Variable Immunodeficiency

Risk Factors of Bronchiectasis in Adult Patients with Common Variable Immunodeficiency

Common Variable Immune Deficiency in Children—Clinical Characteristics Varies Depending on Defect in Peripheral B Cell Maturation

T-Cell Abnormalities in Common Variable Immunodeficiency

Contact Info

Product

Resources

About