Journal of Clinical Immunology

, Volume 39, Issue 6, pp 569–576 | Cite as

Cellular Defects in CVID Patients with Chronic Lung Disease in the USIDNET Registry

  • Erinn S. KellnerEmail author
  • Ramsay Fuleihan
  • Charlotte Cunningham-Rundles
  • The USIDNET Consortium
  • Joshua B. WechslerEmail author
Original Article



Chronic lung disease is the most common cause of morbidity and mortality in patients with common variable immunodeficiency (CVID). While biomarkers exist to predict non-infectious complications, the unique features that define CVID patients with chronic lung disease are not well understood.


We analyzed data from CVID patients from the retrospective USIDNET (United States Immunodeficiency Network) patient database. Patients were categorized into 3 phenotypes for comparison: (1) CVID without chronic lung disease, (2) CVID with bronchiectasis only, and (3) CVID with interstitial lung disease (ILD) with or without bronchiectasis. Among these groups, differences were assessed in demographics, comorbidities, infections, treatments, and peripheral blood immune measures. We analyzed 1518 CVID patients which included 1233 (81.2%) without chronic lung disease, 147 (9.7%) with bronchiectasis only, and 138 (9.1%) with interstitial lung disease.


Patients with ILD had lower CD3+ cell counts (P = .001), CD4+ cell counts (P < .05), and CD8+ cell counts (P < .001) compared with patients without lung disease. Additionally, there was significantly more CVID patients with ILD with pneumonia (P < .001), herpes viruses (P = .01) and fungal infections (P < .001) compared with patients with CVID without chronic lung disease.


This analysis suggests that patients with chronic lung disease may be more likely to have lower peripheral T cell counts and complications of those defects compared with CVID patients without chronic lung disease.


Common variable immunodeficiency CVID granulomatous lymphocytic interstitial lung disease GLILD autoimmunity USIDNET 



We gratefully acknowledge Julie Magnusson, Marla Goldsmith, and Tara Caulder for their continued support with the USIDNET. We also acknowledge and thank all contributing physicians and patients. This work was partially supported by K08DK09772 (to JBW).

Authorship Contributions

ESK: Data collection, data interpretation, and manuscript preparation

RF: Critical review and co-investigator for USIDNET

CCR: Critical review and PI for USIDNET

JBW: Project supervision, data interpretation, and manuscript preparation

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.


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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Allergy and Immunology, Department of MedicineNorthwestern University Feinberg School of MedicineChicagoUSA
  2. 2.Division of Allergy-ImmunologyAnn & Robert H. Lurie Children’s Hospital of ChicagoChicagoUSA
  3. 3.Division of Allergy and Immunology, Department of MedicineThe Icahn School of Medicine at Mount SinaiNew YorkUSA
  4. 4.Division of Gastroenterology, Hepatology & Nutrition, Department of PediatricsAnn & Robert H. Lurie Children’s Hospital of ChicagoChicagoUSA

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