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

doi:10.4329/wjr.v10.i11.172

High-resolution computed tomography findings in humoral primary immunodeficiencies and correlation with pulmonary function tests

World J Radiol. 2018 Nov 28;10(11):172-183. doi: 10.4329/wjr.v10.i11.172.

Authors

Lorenzo Cereser¹, Marco De Carli², Paola d'Angelo³, Elisa Zanelli³, Chiara Zuiani³, Rossano Girometti³

Affiliations

¹ Institute of Radiology, Department of Medicine, University of Udine, Azienda Sanitaria Universitaria Integrata di Udine, Udine 33100, Italy. lcereser@sirm.org.
² Second Unit of Internal Medicine, Azienda Sanitaria Universitaria Integrata di Udine, Udine 33100, Italy.
³ Institute of Radiology, Department of Medicine, University of Udine, Azienda Sanitaria Universitaria Integrata di Udine, Udine 33100, Italy.

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; Common variable immunodeficiency; Immunologic deficiency syndromes; Multidetector computed tomography; Respiratory function tests.