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:
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.
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
Research background

Common variable immunodeficiency disorders (CVID) are the most clinically significant group of humoral primary immunodeficiency diseases (hPIDs), manifesting with recurrent respiratory tract infections and increased susceptibility to autoimmune diseases and malignancy. Other hPIDs are often termed “CVID-like” conditions, and include selective IgA deficiency and isolated IgG subclass deficiency. The initial evaluation of patients newly diagnosed with hPIDs should include high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs).

Research motivation

To the best of our knowledge no previous studies assessed whether HRCT findings differ in terms of prevalence among the two subtypes of hPIDs in adult patients. Demonstrating a difference between CVID and CVID-like patients may influence the time intervals between HRCT examinations during follow-up in these 2 subgroups of hPIDs. Moreover, previous studies assessing a possible correlation between HRCT findings and PFTs results demonstrated contradictory results.

Research objectives

The purpose of this study was twofold: (1) to compare HRCT pulmonary findings in adult patients with different subgroups of hPIDs (i.e., CVID vs CVID-like); and (2) to assess whether HRCT findings predict PFTs results.

Research methods

We included 52 adult patients (38 CVID and 14 CVID-like) who received a definite diagnosis of hPIDs and underwent HRCT and PFTs within one month from the time of diagnosis. One pulmonary radiologist, blinded to patient history and lung function: (1) reviewed the HRCT examinations; (2) recorded two classes of abnormalities, namely airway abnormalities and/or parenchymal-interstitial abnormalities; and (3) scored all abnormalities according to their extension and conspicuity. We calculated the per-patient prevalence of each HRCT abnormality in the overall population and in both subgroups of hPIDs patients, (CVID and CVID-like groups). We performed a logistic regression analysis to assess whether HRCT findings were predictive of a relevant obstructive or restrictive defect at PFTs on the overall study population.

Research results

Of the 52 hPIDs patients, 37 were females and 15 were males, with a mean age of 53.9 ± 12.7 years. We found a high prevalence of HRCT findings (90.4% patients had one or more abnormalities). The prevalence of each of the airway abnormalities considered was not significantly different between the CVID and CVID-like group. Regarding HRCT-detected parenchymal-interstitial abnormalities, the only relevant result was the finding of linear and/or irregular opacities, showing a prevalence of 31.6% in the CVID group and 0 in the CVID-like group, with borderline significance. The presence of tree-in-bud abnormalities was an independent predictor of 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).

Research conclusions

No previous research compared the prevalence of HRCT findings in different subtypes of hPIDs adult patients. After dividing hPIDs patients in CVID vs CVID-like groups, we observed no significant difference in the prevalence of most of airways and parenchymal-interstitial findings between the two groups. This observation supports the hypothesis that these two groups represent comparable hPIDs subtypes, and are candidate to similar management. Tree-in-bud and linear and/or irregular opacities were found to be independent predictors of, respectively, obstructive and restrictive defects on PFTs.

Research perspectives

Our results suggest that morphological assessment with HRCT might be delayed as much as possible to maximize cost-effectiveness and reduce radiation exposure. A possible exception to this might be the case of patients showing tree-in-bud or linear and/or irregular opacities: Scheduling HRCTs at shorter intervals for these patients might provide a reliable morphological counterpart of pulmonary function. Further prospective studies with a proper design are needed to confirm this hypothesis in the follow-up period.