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Cytokine dysregulation despite immunoglobulin replacement therapy in common variable immunodeficiency (CVID)
Remo Poto1,2, Antonio Pecoraro1,2,3, Anne Lise Ferrara1,2
1Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy.
Common variable immunodeficiency (CVID) shows persistent immune dysregulation, with higher cytokine and LPS levels in those with autoimmune disorders. This highlights CVID heterogeneity despite immunoglobulin replacement therapy.
Area of Science:
- Immunology
- Primary Immunodeficiency Disorders
Background:
- Common variable immunodeficiency (CVID) is a prevalent primary immunodeficiency.
- Immunoglobulin replacement therapy (IgRT) manages infections but not underlying immune issues.
Purpose of the Study:
- To evaluate cytokine profiles and inflammatory markers in CVID patients.
- To differentiate immune dysregulation in CVID with infections versus CVID with autoimmune/inflammatory disorders.
Main Methods:
- Measured serum TNF-α, IL-1β, IL-6, IL-10, LPS, and sCD14.
- Compared levels in infectious CVID (INF-CVID), non-infectious CVID (NIC-CVID), and healthy donors (HD).
Main Results:
- Both INF-CVID and NIC-CVID showed elevated cytokines (TNF-α, IL-1β, IL-6, IL-10) versus HD.
- NIC-CVID had significantly higher cytokine elevations, increased LPS, and elevated sCD14 compared to INF-CVID and HD.
Conclusions:
- CVID exhibits persistent cytokine dysregulation even with IgRT.
- Distinct cytokine profiles confirm immune dysregulation heterogeneity within CVID subgroups.
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