Adults with common variable immunodeficiency: a single-center experience*

Muşabak, Uğur; YEŞİLLİK, Fevzi Sait DEMİREL; Baysan, Abdullah; Selcuk, Ali; Kartal, Özgür; Güleç, Mustafa; Öktenli, Çağatay; Şener, Osman

doi:10.3906/sag-1503-22

Cited by 11 publications

(14 citation statements)

References 27 publications

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“…The mean diagnostic delay was 104 ± 91.9 months in the present study, which is consistent with Quinti et al's report (16). Furthermore, Ardeniz et al and Musabak et al also reported similar periods of delayed diagnosis in Turkey (7,8).…”

Section: Discussionsupporting

confidence: 92%

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Risk Factors of Bronchiectasis in Adult Patients with Common Variable Immunodeficiency

Aytekin¹,

Çölkesen²,

Yıldız³

et al. 2019

Asthma Allergy Immunol

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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.

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Section: Discussionsupporting

confidence: 92%

“…In studies conducted in Turkey, Ardeniz et al reported a rate of 30% and Musabak et al reported a rate of 12.9% for consanguineous marriages (7,8). In studies conducted in other countries, Oksenhendler et al and Aghamohammadi et al reported lower rates at 5.4% and 8% , respectively (9,10).…”

Section: Discussionmentioning

confidence: 99%

Risk Factors of Bronchiectasis in Adult Patients with Common Variable Immunodeficiency

Aytekin¹,

Çölkesen²,

Yıldız³

et al. 2019

Asthma Allergy Immunol

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“…Finally, 79 studies met the inclusion criteria and were analyzed. 4 - 14 , 16 - 83 The characteristics of the included studies in this systematic review are depicted in Table 1 . The sample sizes of CVID patients varied from 4 to 2212 in a study from the ESID.…”

Section: Resultsmentioning

confidence: 99%

Infectious Complications Reporting in Common Variable Immunodeficiency: A Systematic Review and Meta-analysis

Zainaldain¹,

Rizvi²,

Rafiemanesh³

et al. 2020

Oman Med J

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Objectives Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by hypogammaglobulinemia and increased susceptibility to recurrent infections. Methods We searched PubMed, Web of Science, and Scopus databases to find eligible studies from the earliest available date to January 2018 with standard keywords. Pooled estimates of the infection prevalence and the corresponding 95% confidence intervals were calculated using random-effects models. Results We found that pneumonia (67.7%) was the most prevalent infection followed by upper respiratory tract (59.0%) and gastrointestinal infections (36.3%). Furthermore, bacterial complications (41.7%) were higher in CVID patients compared to viral (25.4%), parasitic (18.8%), or fungal (3.4%) infections. Patients with longer age at diagnosis presented with fewer disease comorbidities. There was an inverse correlation between T lymphocyte count and viral infections. Moreover, we found that immunoglobulin M (IgM) serum level was inversely correlated with hepatitis C and gastrointestinal infections, and IgG serum level was inversely correlated with infectious arthritis. Higher numbers of CD4 and CD8 T cells were associated with the lower frequencies of otitis media. CVID patients with infections had significantly lower percentages of CD3 T cells. In contrast, higher percentages of CD19 lymphocytes were found in CVID patients who had a history of infections. Conclusions Our findings demonstrated that in addition to hypogammaglobulinemia, patients with CVID have an imbalance in the frequency of T lymphocytes, which is in parallel with the higher frequency of infectious complications.

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“…CVID, as the second-most common primary immunode ciency, presents with a range of manifestations. 1 Skin sequelae are no exception: skin infections occur nearly four times as often in patients with CVID as compared to the general population, with diverse etiologies, including bacterial, viral, and fungal. Patients may also develop autoimmune diseases, such as vitiligo, alopecia areata, and psoriasis.…”

Section: Discussionmentioning

confidence: 99%

“…Due to their de cient immune system, patients with CVID have an increased risk of recurrent respiratory infections, abscesses, pulmonary granulomas, in ammatory bowel disease, autoimmune conditions, and malignancy. 1 However, there is no report on skin abscesses caused by mycoplasma. Herein, we report a case of mycoplasma skin abscesses in a patient whose past medical history raised suspicion for CVID.…”

Section: Introductionmentioning

confidence: 99%

Turning walking pneumonia into recurrent abscesses: a curious case of CVID and review of the literature

Gao

Hussain

Bobber

et al. 2021

Preprint

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Background: common variable immunodeficiency (CVID) is a primary immunodeficiency disorder associated with a broad symptom presentation that is still being characterized. We report a rare case of recurrent mycoplasma skin abscesses in a patient with a history of autoimmune disorders and prolonged mycoplasma pneumonia who was diagnosed with CVID.Case presentation: a 34-year-old woman presented with a history of recurrent abscesses previously confirmed positive for Mycoplasma pneumoniae. Her past medical history of recurrent mycoplasma abscesses, prolonged mycoplasma pneumonia, and autoimmune disorders (mixed connective tissue disease and immune thrombocytopenia) raised suspicion of CVID. Workup included negative anti-mycoplasma antibody titers, hypogammaglobulinemia, and negative anti-pneumococcal antibody titers despite prior vaccination, solidifying the diagnosis of CVID. The patient was discharged on antibiotic and intravenous immunoglobulin therapy with improvement and now follows allergy and immunology long-term for treatment.Conclusions: her diagnostic history underscores the importance of considering the various criteria of CVID for diagnosis, and her unique presentation of M. pneumoniae skin abscesses highlights the broad sequelae patients with CVID can manifest.

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Adults with common variable immunodeficiency: a single-center experience*

Cited by 11 publications

References 27 publications

Risk Factors of Bronchiectasis in Adult Patients with Common Variable Immunodeficiency

Risk Factors of Bronchiectasis in Adult Patients with Common Variable Immunodeficiency

Infectious Complications Reporting in Common Variable Immunodeficiency: A Systematic Review and Meta-analysis

Turning walking pneumonia into recurrent abscesses: a curious case of CVID and review of the literature

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