Published online Nov 28, 2018. doi: 10.4329/wjr.v10.i11.172
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
To compare high-resolution computed tomography (HRCT) findings between humoral primary immunodeficiencies (hPIDs) subtypes; to correlate these findings to pulmonary function tests (PFTs).
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).
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).
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.
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.