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Journal of Clinical Immunology

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01-08-2019 | Bronchiectasis | Original Article

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

Authors: Erinn S. Kellner, Ramsay Fuleihan, Charlotte Cunningham-Rundles, Joshua B. Wechsler, The USIDNET Consortium

Published in: Journal of Clinical Immunology | Issue 6/2019

Abstract

Purpose

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.

Methods

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.

Results

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.

Conclusion

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.

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Title: Cellular Defects in CVID Patients with Chronic Lung Disease in the USIDNET Registry
Authors: Erinn S. Kellner
Ramsay Fuleihan
Charlotte Cunningham-Rundles
Joshua B. Wechsler
The USIDNET Consortium
Publication date: 01-08-2019
Publisher: Springer US
Keywords: Bronchiectasis
Immunodeficiency
Published in: Journal of Clinical Immunology / Issue 6/2019
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-019-00657-w

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

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Conclusion

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