Retrospective Study
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Radiol. Nov 28, 2018; 10(11): 172-183
Published online Nov 28, 2018. doi: 10.4329/wjr.v10.i11.172
High-resolution computed tomography findings in humoral primary immunodeficiencies and correlation with pulmonary function tests
Lorenzo Cereser, Marco De Carli, Paola d’Angelo, Elisa Zanelli, Chiara Zuiani, Rossano Girometti
Lorenzo Cereser, Paola d’Angelo, Elisa Zanelli, Chiara Zuiani, Rossano Girometti, Institute of Radiology, Department of Medicine, University of Udine, Azienda Sanitaria Universitaria Integrata di Udine, Udine 33100, Italy
Marco De Carli, Second Unit of Internal Medicine, Azienda Sanitaria Universitaria Integrata di Udine, Udine 33100, Italy
Paola d’Angelo, Department of Imaging, Bambino Gesù Children's Hospital, IRCCS, Rome 00165, Italy
Author contributions: All authors helped performing the research; Cereser L drafted the concept of the research, designed the study, read the HRCT examinations and wrote the manuscript; De Carli M contributed to designing the study and writing the manuscript; d’Angelo P and Zanelli E collected the data and contributed to analysing the data and writing the manuscript; Zuiani C drafted the concept of the research and contributed to designing the study; Girometti R designed the study, analysed the data and contributed to writing the manuscript.
Institutional review board statement: Our referring Ethical Committee approved this study.
Informed consent statement: By Italian regulations (decision n. 531, December 15, 2016 by the Authority for personal data protection) informed consent acquisition is waived for retrospective studies involving analysis of anonymized data.
Conflict-of-interest statement: All authors declare no conflicts of interest related to this article.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Lorenzo Cereser, MD, Doctor, Institute of Radiology, Department of Medicine, University of Udine, Azienda Sanitaria Universitaria Integrata di Udine, p.le S. Maria della Misericordia 15, Udine 33100, Italy. lcereser@sirm.org
Telephone: +39-432-559266 Fax: +39-432-559867
Received: July 17, 2018
Peer-review started: July 17, 2018
First decision: August 8, 2018
Revised: September 22, 2018
Accepted: October 7, 2018
Article in press: October 7, 2018
Published online: November 28, 2018
Processing time: 146 Days and 14.7 Hours
Abstract
AIM

To compare high-resolution computed tomography (HRCT) findings between humoral primary immunodeficiencies (hPIDs) subtypes; to correlate these findings to pulmonary function tests (PFTs).

METHODS

We retrospectively identified 52 consecutive adult patients with hPIDs who underwent 64-row HRCT and PFTs at the time of diagnosis. On a per-patient basis, an experienced radiologist recorded airway abnormalities (bronchiectasis, airway wall thickening, mucus plugging, tree-in-bud, and air-trapping) and parenchymal-interstitial abnormalities (consolidations, ground-glass opacities, linear and/or irregular opacities, nodules, and bullae/cysts) found on HRCT. The chi-square test was performed to compare the prevalence of each abnormality among patients with different subtypes of hPIDs. Overall logistic regression analysis was performed to assess whether HRCT findings predicted obstructive and/or restrictive PFTs results (absent-to-mild vs moderate-to-severe).

RESULTS

Thirty-eight of the 52 patients with hPIDs showed common variable immunodeficiency disorders (CVID), while the remaining 14 had CVID-like conditions (i.e., 11 had isolated IgG subclass deficiencies and 3 had selective IgA deficiencies). The prevalence of most HRCT abnormalities was not significantly different between CVID and CVID-like patients (P > 0.05), except for linear and/or irregular opacities (prevalence of 31.6% in the CVID group and 0 in the CVID-like group; P = 0.0427). Airway wall thickening was the most frequent HRCT abnormality found in both CVID and CVID-like patients (71% of cases in both groups). The presence of tree-in-bud abnormalities was an independent predictor of moderate-to-severe obstructive defects at PFTs (Odds Ratio, OR, of 18.75, P < 0.05), while the presence of linear and/or irregular opacities was an independent predictor of restrictive defects at PFTs (OR = 13.00; P < 0.05).

CONCLUSION

CVID and CVID-like patients showed similar HRCT findings. Tree-in-bud and linear and/or irregular opacities predicted higher risks of, respectively, obstructive and restrictive defects at PFTs.

Keywords: Bronchiectasis; Multidetector computed tomography; Common variable immunodeficiency; Immunologic deficiency syndromes; Respiratory function tests

Core tip: Humoral primary immunodeficiencies (hPIDs) are a group of conditions characterized by impaired antibody production and presenting with recurrent respiratory infections, autoimmune diseases, and malignancy. Chest high-resolution computed tomography (HRCT) is the imaging technique of choice for detecting, characterizing, and quantifying lung complications in these patients. The aims of this study were to compare HRCT findings in 52 patients with hPIDs subtypes (common variable immunodeficiency disorders - CVID vs CVID-like), and evaluate whether these findings may predict pulmonary function tests results. CVID vs CVID-like patients showed comparable HRCT findings. The presence of tree-in-bud and linear and/or irregular opacities were independent predictors of, respectively, significant obstructive and restrictive defects.