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.
Abstract

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