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