scholarly journals Serum B cell activating factor and interleukin 10 levels in common variable immunodeficiency: Relationship with clinical findings

2019 ◽  
Vol 76 (1) ◽  
pp. 42-49
Author(s):  
Radovan Mijanovic ◽  
Sladjana Andrejevic ◽  
Vladimir Jurisic ◽  
Branka Bonaci-Nikolic
Keyword(s):  
Autoimmune Diseases ◽  
B Cell ◽  
Common Variable Immunodeficiency ◽  
Healthy Subjects ◽  
Serum Levels ◽  
Pulmonary Diseases ◽  
Recurrent Infections ◽  
B Cell Activating Factor ◽  
Chronic Pulmonary Diseases ◽  
Common Variable

Background/Aim. Common variable immunodeficiency (CVID) is an immunologically and clinically heterogeneous disorder. Disturbed cytokine production is implicated in dysfunctional immune response. The aim of this study was to investigated B-cell activating factor (BAFF) and interleukin (IL)- 10 levels in CVID patients. Methods. The study included 28 CVID patients diagnosed and followed during a 20-year period (mean follow-up 14.5 years). Control groups consisted of 4 patients with X-linked agammaglobulinemia (XLA) and 21 healthy subjects. According to clinical characteristics, the CVID patients were divided into four groups which partly overlap: chronic pulmonary diseases (n = 21), splenomegaly (n = 13), autoimmune diseases (n = 9) and patients with recurrent infections despite regular intravenous immunoglobulin (IVIg) substitution (n = 4). The serum levels of BAFF and IL-10 were measured by commercial ELISA. Results. The BAFF levels were found to be higher in all CVID patients compared to the healthy controls (p < 0.01). The most significant differences were observed in the patients with pulmonary diseases and splenomegaly (p < 0.0001). Also, concentrations of IL-10 were higher in all CVID patients in comparison with the XLA patients (p < 0.05) and healthy subjects (p < 0.01). A statistically significant positive correlation (r = 0.86; p < 0.01) was found between the levels of BAFF and IL-10 in the CVID patients with autoimmune diseases. We demonstrated that the CVID patients with chronic pulmonary diseases had higher levels of IL-10, while the CVID patients with recurrent infections had higher BAFF concentrations in comparison to the patients without these features (p < 0.05). Conclusion. In spite of the limited number of patients, this is the first report from Serbia, examining the serum levels of BAFF and IL-10 in the CVID patients. Our study showed significantly increased concentrations of serum BAFF and IL-10 in the patients with CVID compared to the healthy subjects. Further studies are needed to confirm our findings that the BAFF levels are more pronounced in patients with recurrent infections while IL-10 levels are higher in patients with chronic pulmonary diseases.

B Cell-activating Factor and its Targeted Therapy in Autoimmune Diseases

2021 ◽  
Author(s):  
Yidan Zhang ◽  
Jie Tian ◽  
Fan Xiao ◽  
Leting Zheng ◽  
Xiaoxia Zhu ◽  
...  
Keyword(s):  
Targeted Therapy ◽  
Autoimmune Diseases ◽  
B Cell ◽  
B Cell Activating Factor

scholarly journals Expression of TNF-α, April and BCMA in Behcet’s Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Olfat G. Shaker ◽  
Shereen O. Tawfic ◽  
Amira M. El-Tawdy ◽  
Mohamed H. M. El-Komy ◽  
Manal El Menyawi ◽  
...  
Keyword(s):  
Tumor Necrosis Factor ◽  
B Cell ◽  
Behçet’S Disease ◽  
Tumor Necrosis ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Serum Levels ◽  
Behcet's Disease ◽  
B Cell Activating Factor ◽  
Necrosis Factor

Background. Tumor necrosis factor-alpha (TNF-α) is an important proinflammatory cytokine which plays an important role in the immunopathogenesis of Behcet’s disease (BD). B cell activating factor (BAFF) and its homolog A proliferation inducing ligand (APRIL) are members of the tumor necrosis factor family. BAFF binds to 3 receptors, B cell activating factor receptor (BAFF-R), transmembrane activator and calcium modulator ligand interactor (TACI), and B cell maturation antigen (BCMA) that are expressed by B cells.Objective. Estimation of the serum levels of TNF-α, APRIL, BAFF, and BCMA in patients with BD in an effort to evaluate their degree of involvement in the pathogenesis and development of BD.Patients and Methods. This study included 30 male patients fulfilling the international study group criteria for the diagnosis of BD. Twenty age-matched healthy male volunteers served as control. Serum samples were used for quantification of TNF-α, APRIL, BCMA, BAFF, and hsCRP using ELISA techniques.Results. The mean serum levels of TNF-α, APRIL, BCMA, and BAFF were more elevated in cases than in controls in a statistically significant manner(P<0.001). Positive correlation was observed between hs-CRP and BDCAF (Behcet’s disease current activity forum) index (r0.68,P<0.001). None of the TNF family members tested was affected by a positive pathergy test.Conclusions. Patients have significantly higher levels of TNF family members’ (TNF-α, BAFF, APRIL, and BCMA) compared to controls which might contribute to the pathogenesis of BD.

scholarly journals Common variable immunodeficiency disorder associated with bronchiectasis: a case report

2021 ◽  
Vol 8 (3) ◽  
pp. 565
Author(s):  
Mohammad Zahirul Islam Khan ◽  
Kamrul Laila ◽  
Mohammed Mahbubul Islam ◽  
Mohammad Imnul Islam ◽  
Shahana Akhter Rahman
Keyword(s):  
Health Care Providers ◽  
Common Variable Immunodeficiency ◽  
Delayed Diagnosis ◽  
Gene Mutations ◽  
Growth Failure ◽  
Recurrent Infections ◽  
Care Providers ◽  
Immunodeficiency Disorder ◽  
Specific Antibody Production ◽  
Common Variable

Common variable immunodeficiency disorder (CVID) is the commonest type of primary immunodeficiency disorders (PIDs) characterized by hypogammaglobulinemia, defective specific antibody production and increased susceptibility of recurrent infections. Autoimmunity, neoplasm and lymphoproliferative disorders are usually associated with CVID. In most cases, the cause is unknown, but multiple gene mutations (10%) may be associated with CVID. Here, we report an eight years old girl with CVID presented with recurrent infections, growth failure, generalized lymphadenopathy and hepatosplenomegaly. Chest examination and radiological findings of this girl were consistent with bronchiectasis. Lack of awareness among health care providers is the reason for delayed diagnosis of several years for this girl. Therefore, it is essential to raise awareness regarding PID patients among the physicians to improve the quality of life.  

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