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.
Keywords: bronchiectasis; common variable immunodeficiency; pneumonia; primary immunodeficiency.
© 2019 Wiley Periodicals, Inc.