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High-resolution computed tomography findings in humoral primary immunodeficiencies and correlation with pulmonary
Lorenzo Cereser1, Marco De Carli2, Paola d'Angelo3
1Institute of Radiology, Department of Medicine, University of Udine, Azienda Sanitaria Universitaria Integrata di Udine, Udine 33100, Italy. lcereser@sirm.org.
Insights
High-resolution CT scans reveal similar lung abnormalities in common variable immunodeficiency disorders (CVID) and CVID-like conditions. Specific HRCT findings like tree-in-bud predict obstructive lung issues, while linear opacities predict restrictive lung issues.
Area of Science:
- Radiology
- Pulmonology
- Immunology
Background:
- Humoral primary immunodeficiencies (hPIDs) are a group of genetic disorders affecting the immune system's antibody production.
- Pulmonary complications are common in hPIDs, impacting lung function and quality of life.
- High-resolution computed tomography (HRCT) is a key imaging modality for assessing lung involvement in hPIDs.
Purpose of the Study:
- To compare HRCT imaging findings across different subtypes of hPIDs.
- To determine the correlation between specific HRCT abnormalities and pulmonary function test (PFT) results.
- To identify HRCT predictors of obstructive and restrictive lung disease in hPIDs.
Main Methods:
- Retrospective analysis of 52 adult patients with hPIDs who underwent HRCT and PFTs at diagnosis.
- Radiological assessment of airway and parenchymal-interstitial abnormalities on HRCT.
- Statistical comparison of HRCT findings between common variable immunodeficiency disorders (CVID) and CVID-like conditions using chi-square tests.
- Logistic regression analysis to evaluate HRCT findings as predictors of PFT results.
Main Results:
- No significant differences in most HRCT abnormalities were observed between CVID and CVID-like patients (P > 0.05), except for linear and/or irregular opacities (P = 0.0427).
- Airway wall thickening was the most frequent abnormality in both groups (71%).
- Tree-in-bud abnormalities independently predicted moderate-to-severe obstructive defects (OR = 18.75, P < 0.05).
- Linear and/or irregular opacities independently predicted restrictive defects (OR = 13.00, P < 0.05).
Conclusions:
- HRCT findings are largely similar between CVID and CVID-like conditions.
- Specific HRCT patterns, namely tree-in-bud and linear/irregular opacities, are significant predictors of obstructive and restrictive lung function deficits, respectively.
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.
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