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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary radiologic findings in common variable immunodeficiency: clinical and immunological correlations
Paul J Maglione1, Jessica R Overbey2, Lin Radigan1
1Department of Medicine, Division of Clinical Immunology, Icahn School of Medicine at Mount Sinai, New York, New York.
Insights
Interstitial lung disease (ILD) in common variable immunodeficiency (CVID) appears linked to autoimmunity and lymphoproliferation, while bronchiectasis is associated with infection and low CD4+ T-cells. These findings suggest distinct CVID lung disease pathways.
Area of Science:
- Pulmonology
- Immunology
- Radiology
Background:
- The etiology of interstitial lung disease (ILD) in common variable immunodeficiency (CVID) is debated, with possibilities including chronic infection or lymphoid dysregulation.
- Understanding the distinct pathological processes underlying lung manifestations in CVID is crucial for effective management.
Purpose of the Study:
- To elucidate the clinical and immunological factors associated with different lung pathologies in CVID patients.
- To differentiate between infectious and autoimmune/lymphoproliferative drivers of lung disease in CVID.
Main Methods:
- Retrospective analysis of 61 CVID patients' electronic medical records.
- Correlation of chest computerized tomography (CT) findings (bronchiectasis, ground glass opacity, nodules) with clinical and laboratory data.
Main Results:
- Bronchiectasis correlated with low CD4+ T-cell counts, prior pneumonia, and older age.
- Pulmonary nodules associated with higher CD4+:CD8+ ratios, autoimmune conditions, elevated IgM, and younger age.
- Ground glass opacity shared features with nodular disease but also linked to elevated monocytes and liver disease.
Conclusions:
- CT-identified bronchiectasis and ILD (including ground glass opacity and nodules) in CVID correlate with specific clinical and lab markers.
- Bronchiectasis in CVID suggests an infectious etiology with T-cell lymphopenia.
- ILD in CVID points towards autoimmune processes and lymphoproliferation.
Background:
It remains unclear whether interstitial lung disease (ILD) in common variable immunodeficiency (CVID) is a consequence of chronic infection or a manifestation of dysregulated lymphoid proliferation found in those with this condition.
Objective:
To increase understanding of CVID-associated lung disease by comparing clinical and immunologic associations in those with bronchiectasis, ILD, or no lung disease observed on chest computerized tomography (CT).
Methods:
Retrospective review of electronic medical records of 61 patients with CVID was used to identify clinical and laboratory correlates of bronchiectasis, ground glass opacity, and pulmonary nodules on CT scan.
Results:
Significant clinical and immunologic associations were identified for common CT scan findings in CVID. Bronchiectasis was strongly correlated with a CD4+ T-cell count lower than 700 cells/μL and was associated with a history of pneumonia and older age. Pulmonary nodular disease was correlated with increased CD4+:CD8+ T-cell ratios, a history of autoimmune hemolytic anemia or immune thrombocytopenic purpura, elevated IgM, and younger age. Ground glass opacity had similar clinical and laboratory characteristics as those for nodular lung disease but was associated with elevated monocyte counts and the presence of liver disease.
Conclusion:
CT findings of bronchiectasis or ILD, including ground glass opacity and extensive pulmonary nodules, were correlated with selected clinical and laboratory characteristics. These results suggest divergent processes of CVID lung disease, with bronchiectasis more strongly associated with infection and T-cell lymphopenia and ILD more strongly linked with autoimmunity and lymphoproliferation.
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