Risk Factors of Bronchiectasis in Adult Patients  with Common Variable Immunodeficiency

Aytekin, Gökhan; Çölkesen, Fatih; Yıldız, Eray; Arslan, Şevket

doi:10.21911/aai.502

Cited by 4 publications

(4 citation statements)

References 25 publications

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“…Also, thrombocytopenia may be due to splenic sequestration or autoimmunity in addition to immune dysregulation. Our group reported that low platelet count is a risk factor for bronchiectasis in patients with CVID [34]. In our study, platelet values were found to be lower in the group with proteinuria, although not statistically significant.…”

Section: Discussioncontrasting

confidence: 56%

Nephrological factors may cause kidney dysfunction in patients with common variable immunodeficiency

et al. 2021

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Background/aim: Common variable immunodeficiency (CVID) is a heterogeneous primary deficiency characterized by hypogammaglobulinemia, recurrent infections, an increased risk of autoimmune disease, malignancy, and chronic inflammation. Proteinuria is one of the most important prognostic factors causing progression in kidney disease. Proteinuria causes tubulotoxicity, activates inflammatory markers that cause fibrosis, and consequently nephropathy progression. The data is scant in the literature regarding the inflammation and nephropathy in CVID. Hence, in the present study, we aimed to investigate the relationship between tubular dysfunction, proteinuria, and inflammation in patients with CVID. Materials and methods:This was a cross-sectional study involving 27 patients with CVID (15 females, 12 males; mean age, 39.88 ± 13.47 years) and 18 control subjects (10 females, 8 males; mean age, 33.83 ± 7.97 years). Patients were evaluated for kidney functions including glomerular filtration rate, fractional excretion of sodium, metabolic acidosis, serum/urine anion gap, 24-h urine proteinuria and, were grouped in terms of proteinuria. Blood samples obtained from the patients with CVID were taken into 2 mL EDTA tube to evaluate peripheral NK cell subgroups according to CD56 and CD16 expression and CD3, CD4, CD 8 expression to determine subtypes T cells. These cells were evaluated by flow cytometry technique.Results: Urinary density, fractional excretion of sodium, proteinuria, and metabolic acidosis are found to be higher in patients with CVID when compared to healthy controls. In the bivariate correlation analysis, proteinuria was positively correlated with age (r = 0.496, p = < 0.001), CD8+T cells percentage (r = 0.427, p = 0.02). Albumin, CRP, and CD8+T cell percentage were found to be independent variables of proteinuria. Conclusion:Increased chronic ongoing inflammation was found to be associated with proteinuria in patients with CVID. Hence, in routine outpatient clinics, proteinuria should not be overlooked in this group of patients.

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Section: Discussioncontrasting

confidence: 56%

Nephrological factors may cause kidney dysfunction in patients with common variable immunodeficiency

et al. 2021

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“…In the USIDNET (The United States Immunodeficiency Network) registry system, patients with autoimmune cytopenias have been determined to be associated with non-infectious complications, including lymphoproliferation and granulomatous disorders enteropathy, lymphoma, and interstitial pulmonary diseases (16). In another study, reduced platelet count and switched memory B cell ratio was reported to be a risk factor for bronchiectasis (17). Fisher et al (18) reported that autoimmune conditions negatively affect the overall survival rate.…”

Section: Discussionmentioning

confidence: 99%

Low Platelet Level May be a Predictor for Mortality in Adult Patients with Common Variable Immune Deficiency

Aytekin¹,

Çölkesen²,

Yıldız³

et al. 2022

acmr

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Background: Common variable immunodeficiency (CVID) is the most common symptomatic immunodeficiency in adults. We, therefore, aimed to reveal mortality rates, causes of mortality in CVID patients, as well as demographic and clinical characteristics and differences of survived and dead CVID patients Methods: The study group included 50 patients [(Female: 23 (46%), Male: 27 (54%)] with CVID, who were followed up on a regular basis for a period of ten years (115.18 ± 80.74 months).Results: Diagnostic delay was 84 (0-360) months, and the mean follow-up time was 115.18 ± 80.74 months. The most common clinical presenting complaints were frequent and recurrent infections and pneumonia. At diagnosis, serum IgG levels were 1.72 (0.33 -6.90) g/L. The overall survival rate of the patients during the follow-up time was 88%. As a result of univariate Cox regression analysis, platelet count was determined to be an independent risk factor for mortality in CVID patients (Hazard ratio, HR: 0.990, 95% confidence interval, CI: 981-0.999, p: 0.025). When the patients were classified according to mean platelet counts (platelets < 207770/mm3 and platelets > 207770/mm3), the mortality rate in the patient group with platelets < 207770/mm3 was determined to be statistically significantly higher compared to the patient group with platelets > 207770/mm3 (log-rank: 0.013). Conclusions:Clinicians dealing with this patient group should remember that immune dysregulation and low platelet count are independent risk factors for mortality and they should remarkably follow up patients with low platelet count closely.

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“…Elevated levels of ALP are usually reduced to a normal range by Bone mineral loss is a well-defined complication in chronic lung diseases (26). Bronchiectasis and chronic lung disease may develop due to the frequent respiratory infections in patients with CVID, and lung function loss may occur due to granulomatous lung disease and interstitial lung involvement (27). In addition, patients who develop chronic lung disease use a higher dose of immunosuppressants to control their respiratory complaints and are immobilized, which may lead to bone mineral loss (26,28).…”

Section: Discussionmentioning

confidence: 99%

Bone Metabolism Alterations in Patients with Common Variable Immune Deficiency: A Retrospective Cohort Study

Aytekin¹,

Çölkesen²,

Yıldız³

et al. 2020

Asthma Allergy Immunol

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Objective: Common variable immunodeficiency (CVID) is a relatively frequent primary immunodeficiency disorder characterized by impaired B cell differentiation with hypogammaglobulinemia in the European Society for Immunodeficiencies (ESID) registry system. Increased inflammatory cytokines, prolonged and/or recurrent infections, gastrointestinal complications, and anti-inflammatory medications are risk factors for diminished bone mineral density (BMD) in CVID patients. Materials and Methods: The study group included 32 patients with CVID (19 males and 13 females; mean age: 37.33 ± 13.70 years, 40.6% female), who had been followed up on a regular basis for a period of four years. The patients were categorized into two groups according to their BMD: low BMD and normal BMD. Results: Seventeen patients (8 females and 9 males) had normal BMD (mean age 34.94 ± 11.41 years, 47.1% female) and fifteen patients (5 females and 10 males) had low BMD (mean age 40.03 ± 15.87 years, 33.3% female). In the low BMD group, three patients had osteoporosis and 12 patients had osteopenia. Univariate regression analysis revealed that lymphopenia (odds ratio, OR:6.562, 95% confidence interval, CI: 1.095-39.324, p=0.039) was significantly associated with low BMD. Multivariate regression analysis showed that higher alkaline phosphatase (ALP) levels (OR:1.017, 95% CI 1.001-1.033, p=0.041), lymphopenia (OR:11.028, 95% CI 1.326-91.709, p=0.026), and lower folic acid levels (OR:1.284, 95% CI 1.007-1.637, p=0.043) were also independent predictors for low BMD. Conclusion: Even with some limitations such as the small number for the study population, a single center experience, and a crosssectional design, we recommend that clinical immunologists should be alert for diminished BMD in CVID patients, especially those with low folic acid and high ALP levels and lymphopenia.

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Risk Factors of Bronchiectasis in Adult Patients with Common Variable Immunodeficiency

Cited by 4 publications

References 25 publications

Nephrological factors may cause kidney dysfunction in patients with common variable immunodeficiency

Nephrological factors may cause kidney dysfunction in patients with common variable immunodeficiency

Low Platelet Level May be a Predictor for Mortality in Adult Patients with Common Variable Immune Deficiency

Bone Metabolism Alterations in Patients with Common Variable Immune Deficiency: A Retrospective Cohort Study

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