Common variable immunodeficiency (CVID) is a heterogeneous group of disorders, characterized by recurrent upper and lower respiratory tract infections and some non-infectious clinical complications.
The current study aimed to provide a detailed evaluation of respiratory presentations and complications in a cohort of Iranian patients with CVID.
The present retrospective cohort study was conducted on 245 CVID patients who were recorded at the Iranian primary immunodeficiency disorders registry network. Respiratory manifestations were evaluated by reviewing clinical hospital records, immunologic findings, pulmonary function tests (PFT), and high-resolution computed tomography (HRCT) scans.
The majority of patients (n=208, 85.2%) had experienced at least one episode of acute respiratory manifestation and pneumonia was observed in 31.6 % (n=77) of cases as a first disease manifestation. During the follow-up pneumonia, sinusitis and otitis media were documented in 166 (68.6%), 125 (51.2%) and 103 (42.6%) cases, respectively. Abnormal PFT measurements were documented in 53.8 % of patients. Among these patients, 21.5% showed restrictive changes, whereas 18.4% of patients showed an obstructive pattern. Bronchiectasis was the most frequent radiological finding confirmed in 27.2 % of patients. Patients with bronchiectasis were older at the time of immunodeficiency diagnosis ( p <0.001) and had longer diagnosis delay ( p <0.001) when compared to patients without bronchiectasis.
This study highlights the importance of monitoring the respiratory tract system even in asymptomatic patients. PFT and CT scans are the most commonly used techniques aiming to identify these patients early aiming to reduce the rate of long-term respiratory complications.
Copyright © 2020. Published by Elsevier Inc.