scholarly journals Oxidative stress in common variable immunodeficiency

2021 ◽  
Vol 19 ◽  
pp. 205873922110024
Author(s):  
Sevgen Tanir Basaranoglu ◽  
Sukru Cekic ◽  
Emine Kirhan ◽  
Melahat Dirican ◽  
Sara S. Kilic
Keyword(s):  
Oxidative Stress ◽  
Common Variable Immunodeficiency ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Infectious Complications ◽  
Clinical Syndrome ◽  
University Hospital ◽  
Unknown Etiology ◽  
Healthy Controls ◽  
Oxidative Stress Biomarkers ◽  
Common Variable

Common variable immunodeficiency (CVID) is a heterogenous group of immunologic disorders of unknown etiology. Alterations of the normal cellular balance due to an increase in reactive oxygen species and/or decrease in antioxidant defense may lead to increased oxidative stress. We aimed to evaluate the levels of oxidative stress biomarkers in patients with CVID who had different presentations. We investigated the serum catalase (CAT), erythrocyte superoxide dismutase (SOD), erythrocyte reduced glutathione as antioxidants and serum malondialdehyde levels as lipid peroxidation marker in patients with CVID in Uludag University Hospital Department of Pediatric Allergy and Immunology’s outpatient clinics. In the analysis, there were 21 patients and 27 matched healthy controls. The median levels of CAT in patients with CVID was significantly lower than in healthy controls ( p = 0.04). Among the patients with CVID, 19% had autoimmune disease, one had Sjögren’s syndrome, one had autoimmune alopecia, one had juvenile rheumatoid arthritis, and one had chronic inflammatory demyelinating polyneuropathy. Patients with autoimmune complications had significantly lower CAT levels compared to the ones without autoimmune diseases ( p = 0.03). The patients without non-infectious complications (NICs) had lower SOD levels than the patients with NICs ( p = 0.05). The analysis of oxidative stress markers in the patients with CVID suggested a series of abnormalities in the anti-oxidant system. The clinical syndrome associations may be a useful tool for future studies to set prediction markers for the prognosis of patients with CVID.

scholarly journals Common variable immunodeficiency patients display elevated plasma levels of granulocyte activation markers elastase and myeloperoxidase

2019 ◽  
Vol 33 ◽  
pp. 205873841984338 ◽  
Author(s):  
Jiří Litzman ◽  
Zita Chovancová ◽  
Petr Bejdák ◽  
Marek Litzman ◽  
Zdeněk Hel ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Plasma Levels ◽  
Cell Activation ◽  
Acute Infection ◽  
Stable State ◽  
Enzyme Linked Immunosorbent Assay ◽  
Healthy Controls ◽  
Activation Markers ◽  
Common Variable ◽  
Ivig Administration

Common variable immunodeficiency disorders (CVIDs) represent a group of primary immunodeficiency diseases characterized by hypogammaglobulinemia and dysfunctional immune response to invading pathogens. Previous studies have indicated that CVID is associated with microbial translocation and systemic myeloid cell activation. The goal of this study was to determine whether patients with CVID display elevated systemic levels of markers of granulocyte activation and whether the levels are further influenced by intravenous immunoglobulin (IVIg) infusions. The plasma levels of granulocyte activation markers elastase and myeloperoxidase were determined using enzyme-linked immunosorbent assay (ELISA) in 46 CVID patients and 44 healthy controls. All CVID patients were in a stable state with no apparent acute infection. In addition, granulocyte activation markers’ plasma levels in 24 CVID patients were determined prior to and 1 h following IVIg administration. Neutrophil elastase and myeloperoxidase plasma levels were significantly higher in CVID patients than in healthy controls. Systemic elastase levels were further increased following IVIg administration. In vitro stimulation of 13 CVID patients’ whole blood using IVIg in a therapeutically relevant dose for 2 h resulted in a significant increase in plasma elastase levels compared to unstimulated blood. The data presented here indicate that CVID is associated with chronic granulocytic activation which is further exacerbated by administering IVIg. Increased myeloperoxidase and elastase levels may contribute to associated comorbidities in CVID patients.

scholarly journals Increased Expression of B Lymphocyte Induced Maturation Protein 1 (BLIMP1) in Patients with Common Variable Immunodeficiency (CVID)

2020 ◽  
Author(s):  
Shockrollah Farrokhi ◽  
Faezeh Abbasi-rad ◽  
Nafiseh Esmaeil ◽  
Roya Sherkat ◽  
Reza Yazdani ◽  
...  
Keyword(s):  
B Cells ◽  
Protein Expression ◽  
B Cell ◽  
Common Variable Immunodeficiency ◽  
B Lymphocyte ◽  
Plasma Cells ◽  
Healthy Controls ◽  
Maturation Protein ◽  
Mrna And Protein Expression ◽  
Common Variable

Common variable immunodeficiency (CVID) is a primary immune deficiency disorder characterized by a failure in B cell differentiation, impaired immunoglobulin production, and defect in response to vaccines. As a result of defective B cell maturation and differentiation in CVID, the affected patients commonly present with reduced numbers of memory B cell and antibody-secreting plasma cells. B-cell lymphoma 6 protein (BCL6) and B lymphocyte induced maturation protein 1 (BLIMP1) molecules are two important transcription factors that have key roles in the maturation of B cells to plasma cells. Hence, in the current survey, we aimed to evaluate the mRNA and protein expression levels of BCL6 and BLIMP1 in B lymphocytes isolated from peripheral blood in CVID patients. We collected blood samples from 12 CVID patients and 12 healthy controls. We isolated peripheral blood mononuclear cells (PBMCs) using Ficoll density gradient separation. Then, CD19+ B cells were purified using MACS. The protein expression and transcriptional level of BCL6 and BLIMP1 were respectively measured using flow cytometry and real-time PCR. Our results showed that the BLIMP1 mRNA expression, as well as BLIMP1 protein expression, were significantly higher in CVID patients compared to control subjects (p=0.009 and p=0.007, respectively). However, we found no significant difference in mRNA and protein expression of BCL6 between patients and healthy controls. According to our findings, increased mRNA and protein expression levels of BLIMP1 could be involved in defective maturation of B cells in patients with CVID and elucidate mechanistic insights into the pathogenesis of this disorder.

Granulomatous lymphocytic interstitial lung disease as a complication of common variable immunodeficiency

2021 ◽  
Vol 31 (6) ◽  
pp. 816-821
Author(s):  
Alexandr V. Averyanov ◽  
Anastasia S. Perkina
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Common Variable Immunodeficiency ◽  
Clinical Case ◽  
Infectious Complications ◽  
Pathological Process ◽  
Malignant Neoplasms ◽  
Recurrent Infections ◽  
Autoimmune Conditions ◽  
Common Variable

Common variable immunodeficiency (CVID) is a rare immunodeficiency, the classic manifestation being recurrent infections. Other lesions are often found in CVID patients, such as malignant neoplasms, autoimmune conditions caused by abnormal cellular immunity, in addition to infectious complications. Usually, the pathological process involves the lungs. This article presents a clinical case of a patient with CVID complicated by granulomatous lymphocytic interstitial lung disease.

scholarly journals Involvement of miR-142 and miR-155 in Non-Infectious Complications of CVID

Molecules ◽  
2020 ◽  
Vol 25 (20) ◽  
pp. 4760
Author(s):  
Giuliana Amato ◽  
Federica Vita ◽  
Paolina Quattrocchi ◽  
Paola Lucia Minciullo ◽  
Giovanni Pioggia ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Bacterial Infections ◽  
Infectious Complications ◽  
Knock Out ◽  
Scientific Databases ◽  
Clinical Complications ◽  
Mesh Terms ◽  
Bibliographic Search ◽  
Epigenetic Phenomenon ◽  
Common Variable

Background and objectives: Common variable immunodeficiency (CVID) is the most prevalent antibody impairment. It is characterized by failure in immunoglobulin and protective antibody generation and defined by an increased tendency toward bacterial infections, autoimmunity, and malignancy. Most CVID diagnoses do not follow a classical Mendelian pattern of inheritance. In recent years, CVID has been considered an epigenetic phenomenon in the majority of cases, overtaking previous monogenetic and/or polygenetic theories. The aim of this study was to review the role of microRNAs (miRNAs) in CVID, focusing on the involvement of the same miRNAs in various non-infectious clinical complications of CVID, mainly autoimmunity and/or cancer. Materials and Methods: A bibliographic search of the scientific literature was carried out independently by two researchers in scientific databases and search engines. The MeSH terms “microRNAs” and “common variable immunodeficiency” were used. All research articles from inception to May 2020 were considered. Results: The literature data showed the involvement of two miRNAs in primary immunodeficiency: miR-142 and miR-155. Both of these miRNAs have been investigated through mice models, in which miR-142 and miR-155 were deleted. These knock-out (KO) mice models showed phenotypic analogies to CVID patients with hypogammaglobulinemia, adaptive immunodeficiency, polyclonal proliferation, lung disease, and enteric inflammation. miR-142 and miR-155 have been found to be involved in the following autoimmune and neoplastic clinical complications of CVID: Gastric cancer, gastric mucosa-associated lymphoid tissue (MALT) lymphoma, natural killer/Tcell lymphoma (NKTCL), and immune thrombocytopenia. Conclusions: miR-142 and miR-155 deregulation leads to similar CVID phenotypesin KO mice models. Although no data are available on the involvement of these miRNAs in human CVID, their dysregulation has been detected in human CVID comorbidities. The literature data show that miRNA sequences in murine models are comparable to those in humans; therefore, miR-142 and miR-155 involvement in human CVID could be hypothesized.

scholarly journals Persistent activation of the tumor necrosis factor system in a subgroup of patients with common variable immunodeficiency--possible immunologic and clinical consequences

Blood ◽  
1996 ◽  
Vol 87 (2) ◽  
pp. 674-681 ◽  
Author(s):  
P Aukrust ◽  
E Lien ◽  
AK Kristoffersen ◽  
F Muller ◽  
CJ Haug ◽  
...  
Keyword(s):  
Tumor Necrosis Factor ◽  
Common Variable Immunodeficiency ◽  
Peripheral Blood ◽  
Tumor Necrosis ◽  
Mononuclear Cells ◽  
Tnf Alpha ◽  
Healthy Controls ◽  
Necrosis Factor ◽  
Common Variable

In patients with common variable immunodeficiency (CVI), we have previously defined a subgroup of patients (CVIHyper) characterized by decreased numbers of CD4+ lymphocytes in peripheral blood, splenomegaly, and persistent immune activation in vivo, particularly of monocytes/macrophages. To further characterize this hyperactivity, parameters of activation of the tumor necrosis factor (TNF) system (TNF alpha and soluble TNF receptors [sTNFRs]) were measured in 24 patients with CVI and 20 healthy controls. Patients with CVI had significantly higher serum levels of TNF alpha and both types of sTNFRs, with the highest levels in the CVIHyper subgroup. In vitro, peripheral blood mononuclear cells (PBMC) and purified monocytes from CVIHyper patients spontaneously released significantly higher levels, and, after lipopolysaccharide (LPS) stimulation, significantly lower levels of TNF alpha and soluble p75-TNFR than cells from both other CVI patients and healthy controls. CVIHyper patients also had significantly higher TNF alpha:sTNFRs ratios in both serum and in unstimulated PMBC supernatants. The present study demonstrates persistent in vivo activation of the TNF system in CVI, particularly in the CVIHyper subgroup. This activation may contribute to the pathogenesis of both clinical and immunologic manifestations in CVI.

scholarly journals Selenium-Related Nutritional Status in Patients With Common Variable Immunodeficiency: Association With Oxidative Stress and Atherosclerosis Risk

2021 ◽  
Author(s):  
Itana Andrade ◽  
Fabíola Suano-Souza ◽  
Fernando Fonseca ◽  
Carolina Lago ◽  
Roseli Sarni
Keyword(s):  
Oxidative Stress ◽  
Nutritional Status ◽  
Chronic Diseases ◽  
Common Variable Immunodeficiency ◽  
Positive Impact ◽  
Clinical Care ◽  
Chronic Infections ◽  
Inborn Errors ◽  
Atherosclerosis Risk ◽  
Common Variable

Abstract Background: Common variable immunodeficiency (CVID) is an innate immunity error, possibly associated with recurrent or chronic infections and autoimmune / inflammatory diseases and neoplasms. It is suggested that these conditions lead to persistent immune stimulation and increased oxidative stress. A positive impact on the survival of patients with an inborn errors of immunity was observed with advanced clinical care protocols, thus raising concerns about the risk of developing other associated chronic diseases, such as atherosclerosis. Studies suggest that selenium (Se) is a protective trace element against damage caused by oxidative stress. Thus, it is postulated that adequate consumption reduces the risk of some chronic diseases. Results: The median age of CVID patients was 36.8 years, with a female predominance. Low concentrations of GPx, Se and apo A-1 were observed in the patients, besides the presence of dyslipidemia and higher concentrations of adiponectin, us-CRP, and LDLox. There was no association between the concentrations of Se and GPx and the biomarkers of lipid metabolism involved in atherosclerosis risk, except for a positive association with apo A-1 and HDL. The median of polyunsaturated fat was lower in CVID patients and the intake of zinc and retinol was higher among them when compared to controls. Conclusion: The study showed a higher risk of cardiovascular disease in CVID patients. The presence of low selenium in CVID patients points to the importance of assessing the selenium-related nutritional status in these patients.

scholarly journals Total Antioxidant Capacity, Catalase Activity and Salivary Oxidative Parameters in Patients with Temporomandibular Disorders

2020 ◽  
Author(s):  
Neda Omidpanah ◽  
Saba Ebrahimi ◽  
Asad Vaisi Raygani ◽  
Hadi Mozafari ◽  
Mansour Rezaei
Keyword(s):  
Oxidative Stress ◽  
Antioxidant Capacity ◽  
Temporomandibular Disorders ◽  
Catalase Activity ◽  
Total Antioxidant Capacity ◽  
Control Group ◽  
Healthy Controls ◽  
Oxidative Stress Biomarkers ◽  
Reducing Ability ◽  
Total Antioxidant

Objectives: Temporomandibular disorders (TMD) are characterized by pain or discomfort in the temporomandibular joint, periauricular region, masticatory muscles, and neck on one or both sides. It may also be associated with joint sounds, restricted mandibular movements and mandibular deviation. Oxidative agents may have a deleterious role in the pathogenesis of joint diseases, and oxidative stress can lead to TMD. The aim of this study was to assess the oxidative stress biomarkers in the saliva of TMD patients and healthy controls. Materials and Methods: This case-control study was conducted on 30 patients with TMDs (5 males and 25 females) with a mean age of 30.7±13.2 years, and 30 healthy controls (5 males and 25 females) with a mean age of 29.16±11.2 years. Saliva samples were collected according to the standard protocol and the total antioxidant capacity of the saliva (non-enzymatic), catalase activity, and malondialdehyde (MDA) levels were measured using the ferric reducing ability of plasma, Aebi’s method, and high-performance liquid chromatography, respectively. Finally, The MDA levels were analyzed by the Mann-Whitney test. Other quantitative parameters were analyzed by independent t-test.  Results: TMD patients had significantly higher salivary levels of MDA compared to the control group (P=0.001). But there were no significant differences in catalase (P=0.49) and total antioxidant capacity (P=0.22) of TMD patients and healthy controls. Conclusion: It seems that oxidative stress may be involved in the pathogenesis of TMDs.

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