Two Sides of the Same Coin: Pediatric-Onset and Adult-Onset Common Variable Immune Deficiency

Sanchez, Lauren A.; Maggadóttir, Sólrún Melkorka; Pantell, Matthew S.; Lugar, Patricia L.; Rundles, Charlotte Cunningham; Sullivan, Kathleen E.

doi:10.1007/s10875-017-0415-5

Cited by 35 publications

(32 citation statements)

References 23 publications

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“…Our results showed that in patients with bronchiectasis, the frequency of splenomegaly, allergy and asthma and otitis media was higher in pediatric‐onset than the patients with adult‐onset CVID. Nevertheless, in a report by Sanchez et al, the frequency of bronchiectasis (12.4% vs 20.8%), ILD (4.2% vs 9.9%), and pulmonary nodules (1.6% vs 3.4%) was lower in pediatric‐onset than adult‐onset CVID. It was reported that patients with CVID and parental consanguinity that diagnosed in childhood are more severely affected by autoimmunity, inflammatory lung disease, and enteropathy than adults diagnosed later in life .…”

Section: Discussionmentioning

confidence: 89%

“…Nevertheless, in a report by Sanchez et al, the frequency of bronchiectasis (12.4% vs 20.8%), ILD (4.2% vs 9.9%), and pulmonary nodules (1.6% vs 3.4%) was lower in pediatric‐onset than adult‐onset CVID. It was reported that patients with CVID and parental consanguinity that diagnosed in childhood are more severely affected by autoimmunity, inflammatory lung disease, and enteropathy than adults diagnosed later in life . In addition, the patients with early‐onset CVID are more likely to have a monogenetic defect (such as, LRBA, CTLA‐4, PIK3CD) that may lead to bronchiectasis to due severe immune dysregulations that are associated with an inflammatory response in the lung .…”

Section: Discussionmentioning

confidence: 89%

“…It was reported that patients with CVID and parental consanguinity that diagnosed in childhood are more severely affected by autoimmunity, inflammatory lung disease, and enteropathy than adults diagnosed later in life. 21,22 In addition, the patients with earlyonset CVID are more likely to have a monogenetic defect (such as, LRBA, CTLA-4, PIK3CD) that may lead to bronchiectasis to due severe immune dysregulations that are associated with an inflammatory response in the lung. [23][24][25][26] This may be different from the development of bronchiectasis in patients with adult-onset CVID, in which the etiology may be more chronic and patients may less often have inflammatory lung disease.…”

Section: Discussionmentioning

confidence: 99%

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Bronchiectasis in common variable immunodeficiency: A systematic review and meta‐analysis

Ramzi

Jamee

Bakhtiyari

et al. 2019

Pediatric Pulmonology

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Background Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiency disorder characterized by infectious and noninfectious complications. Bronchiectasis continues to be a common respiratory problem and therapeutic challenge in CVID. The aim of this study is to estimate the overall prevalence of bronchiectasis and its associated phenotype in patients with CVID. Methods A systematic literature search was performed in Web of Science, PubMed, and Scopus from the earliest available date to February 2019 with standard keywords. All pooled analyses of bronchiectasis prevalence and the corresponding 95% confidence intervals (CIs) were based on random‐effects models. Results Fifty‐five studies comprising 8535 patients with CVID were included in the meta‐analysis. Overall prevalence of bronchiectasis was 34% (95% CI: 30‐38; I2 = 90.19%). CVID patients with bronchiectasis had significantly lower serum immunoglobulin A (IgA) and IgM levels at the time of diagnosis compared with those without bronchiectasis. Among the clinical features, the frequencies of splenomegaly, pneumonia, otitis media, and lymphocytic interstitial pneumonia were significantly higher in CVID patients with bronchiectasis compared with those without bronchiectasis, respectively. Conclusion A higher prevalence of bronchiectasis in patients with CVID should be managed by controlling recurrent and severe pneumonia episodes which are immune dysregulation since this complication is associated with poor prognosis in these patients.

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Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 99%

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Bronchiectasis in common variable immunodeficiency: A systematic review and meta‐analysis

Ramzi

Jamee

Bakhtiyari

et al. 2019

Pediatric Pulmonology

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“…Pediatric‐onset CVID patients had more frequent episodes of otitis media, developmental delay, and failure‐to‐thrive as compared to patients with adult‐onset CVID. Adult CVID patients were more frequently diagnosed with bronchitis, arthritis, depression, and fatigue; they also had somewhat more frequent diagnoses of autoimmunity, lymphoma and other malignancies . In all series, patients in their fifth or later decades have also been reported.…”

Section: Demographicsmentioning

confidence: 92%

“…Adult CVID patients were more frequently diagnosed with bronchitis, arthritis, depression, and fatigue; they also had somewhat more frequent diagnoses of autoimmunity, lymphoma and other malignancies. 26 In all series, patients in their fifth or later decades have also been reported. The causes of B-cell failure in older adults are unclear, but the most of the genetic defects described below, can also be found in these subjects.…”

Section: Demog R Aphic Smentioning

confidence: 99%

Common variable immune deficiency: Dissection of the variable

Cunningham‐Rundles

2018

Immunological Reviews

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Summary Starting about 60 years ago, a number of reports appeared that outlined the severe clinical course of a few adult subjects with profound hypogammaglobinemia. Puzzled by the lack of family history and adult onset of symptoms in most, the name “acquired” hypogammaglobinemia was given, but later altered to the current name common variable immune deficiency (CVID). Pathology reports remarked on the loss of lymph node architecture and paucity of plasma cells in lymphoid tissues in these subjects. While characterized by reduced serum IgG and IgA and often IgM, and thus classified amongst the B cell defects, an increasing number of cellular defects in these patients have been recognized over time. In the early years, severe respiratory tract infections commonly led to a shortened life span, but the wide spread availability of immune globulin concentrates for the last 25 years has improved survival. However, chronic non-infectious inflammatory and autoimmune conditions have now emerged as challenging clinical problems; these require further immunologic understanding and additional therapeutic measures. Recent study of this phenotypic syndrome have provided an increasingly fertile ground for the identification of autosomal recessive and now more commonly, autosomal dominant gene defects which lead to the loss of B cell development in this syndrome.

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Influenza‐specific IgG1⁺ memory B‐cell numbers increase upon booster vaccination in healthy adults but not in patients with predominantly antibody deficiency

et al. 2020

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Background. Annual influenza vaccination is recommended to all individuals over 6 months of age, including predominantly antibody deficiency (PAD) patients. Vaccination responses are typically evaluated by serology, and because PAD patients are by definition impaired in generating IgG and receive immunoglobulin replacement therapy (IgRT), it remains unclear whether they can mount an antigen-specific response. Objective. To quantify and characterise the antigen-specific memory B (Bmem) cell compartment in healthy controls and PAD patients following an influenza booster vaccination. Methods. Recombinant hemagglutinin (HA) from the A/Michigan/2015 H1N1 (AM15) strain with an AviTag was generated in a mammalian cell line, and following targeted biotinylation, was tetramerised with BUV395 or BUV737 streptavidin conjugates. Multicolour flow cytometry was applied on blood samples before and 28 days after booster influenza vaccination in 16 healthy controls and five PAD patients with circulating Bmem cells. Results. Recombinant HA tetramers were specifically recognised by 0.5-1% of B cells in previously vaccinated healthy adults. HA-specific Bmem cell numbers were significantly increased following booster vaccination and predominantly expressed IgG1. Similarly, PAD patients carried HAspecific Bmem cells, predominantly expressing IgG1. However, these numbers were lower than in controls and did not increase following booster vaccination. Conclusion. We have successfully identified AM15-specific Bmem cells in healthy controls and PAD patients. The presence of antigen-specific Bmem cells could offer

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Two Sides of the Same Coin: Pediatric-Onset and Adult-Onset Common Variable Immune Deficiency

Abstract: These results suggest that pediatric CVID is not a distinct phenotype. Major features were comparable across the groups. This study underscores the need for continued longitudinal study of pediatric and early-onset CVID patients to further characterize accrual of features over time.

Cited by 35 publications

References 23 publications

Bronchiectasis in common variable immunodeficiency: A systematic review and meta‐analysis

Bronchiectasis in common variable immunodeficiency: A systematic review and meta‐analysis

Common variable immune deficiency: Dissection of the variable

Influenza‐specific IgG1⁺ memory B‐cell numbers increase upon booster vaccination in healthy adults but not in patients with predominantly antibody deficiency

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Two Sides of the Same Coin: Pediatric-Onset and Adult-Onset Common Variable Immune Deficiency

Abstract: These results suggest that pediatric CVID is not a distinct phenotype. Major features were comparable across the groups. This study underscores the need for continued longitudinal study of pediatric and early-onset CVID patients to further characterize accrual of features over time.

Cited by 35 publications

References 23 publications

Bronchiectasis in common variable immunodeficiency: A systematic review and meta‐analysis

Bronchiectasis in common variable immunodeficiency: A systematic review and meta‐analysis

Common variable immune deficiency: Dissection of the variable

Influenza‐specific IgG1+ memory B‐cell numbers increase upon booster vaccination in healthy adults but not in patients with predominantly antibody deficiency

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Resources

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Influenza‐specific IgG1⁺ memory B‐cell numbers increase upon booster vaccination in healthy adults but not in patients with predominantly antibody deficiency