Bronchial Asthma and Bronchial Hyperresponsiveness and Their Characteristics in Patients with Common Variable Immunodeficiency

Abstract

Background: Common variable immunodeficiency (CVID) is one of the most frequent primary immunodeficiencies and is characterized by disturbed immunoglobulin production and dysregulation of the immune system. Results of previous studies suggest a higher prevalence of bronchial asthma (BA) in CVID patients than in the general population. We initiated this study to evaluate lung functions and identify risk factors for BA and bronchial hyperresponsiveness (BHR) in patients with CVID. Methods: Twenty-three patients with CVID were included in this study. In all of them, spirometry and a metacholine bronchoprovocation test were performed. We also investigated the role of atopy, eosinophilic inflammation, and potential risk factors such as gender, age, or immunoglobulin levels at the time of diagnosis. Results: BHR was confirmed in 12 patients (52%), all of whom had normal FEV₁ and FEV₁/FVC. However, BHR-positive patients had significantly decreased MEF25. BHR-positive patients had also more symptoms related to bronchial obstruction, with 8 of them (35%) being suspected of having BA at the end of the study. A higher prevalence of BHR was found in females, with a relative risk of 2.89. Conclusions: An increased prevalence of BHR and BA was detected in CVID patients compared to the general population. BA may develop despite the disturbed immunoglobulin production, and the majority of patients display nonatopic and noneosinophilic properties. These results suggest a limited role of atopy and eosinophilic inflammation in the pathogenesis of BA in CVID patients.

Transgenic overexpression of α7 integrin in smooth muscle attenuates allergen‐induced airway inflammation in a murine model of asthma

“…Thus, prolonged inflammation due to allergen exposure can lead to airway remodeling and promote declining lung function. 33 , 34 , 35 , 36 , 37 In this study, we characterized the effect of smooth muscle‐specific overexpression of α7 integrin on airway inflammation and resistance using acute and chronic mouse models of allergic asthma. The effect of α7 integrin downregulation in asthmatic smooth muscle in vitro has been demonstrated.…”

“…Allergic asthma is associated with leukocyte infiltration and the release of an array of cytokines and chemokines that modulate ASM phenotype. Thus, prolonged inflammation due to allergen exposure can lead to airway remodeling and promote declining lung function 33–37 . In this study, we characterized the effect of smooth muscle‐specific overexpression of α7 integrin on airway inflammation and resistance using acute and chronic mouse models of allergic asthma.…”

Evaluation of pulmonary findings in patients with humoral immune deficiency

“…Mukozal immünitede meydana gelen defekt inhalen alerjen duyarlılığını, bronşiyal hiperreaktiviteyi ve sonuç olarak da astım gelişimini kolaylaştırır (19). Yaygın değişken immün yetmezlikli olgularda astım patogenezi hala açık değildir, çoğunlukla intrinsik astım (non atopik) görülmektedir (20). Çeşitli çalışmalarda YDİY'li olgularda astım sıklığı %9-15 oranında bildirilmiştir (15,(21)(22)(23).…”

“…The defect that occurs in mucosal immunity facilitates inhaled allergen sensitivity, bronchial hyperreactivity, and thus development of asthma ( 19 ). The pathogenesis of asthma in patients with common variable immunodeficiency is still not clear; intrinsic asthma (non-atopic) is observed mostly ( 20 ). In different studies, the frequency of asthma has been reported to range between 9% and 15% in patients with CVID ( 15 , 21 – 23 ).…”

Organ‐based clues for diagnosis of inborn errors of immunity: A practical guide for clinicians

“… 6 , 7 , 8 Moreover, asthma can provide clues to the diagnosis of cartilage‐hair hypoplasia (CHH), CVID, SIgAD, DOCK8 deficiency, and selective IgM deficiency (SIgMD). 9 , 10 , 11 , 12 , 13 Chronic cough and pleurisy are noticed in CVID, X‐linked agammaglobulinemia (XLA), and SIgAD. 14…”