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B-cell activating factor (BAFF) is elevated in chronic granulomatous disease
Kabir Matharu1, Kol A Zarember, Beatriz E Marciano
1Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
Clinical Immunology (Orlando, Fla.)
|June 19, 2013
Summary
Chronic Granulomatous Disease (CGD) involves increased B-cell activating factor (BAFF) due to inflammation. This study found elevated BAFF in CGD patients, linked to inflammatory markers and reduced B-cell receptor expression.
Area of Science:
- Immunology
- Genetics
- Inflammation Biology
Background:
- Chronic Granulomatous Disease (CGD) is an inherited immune disorder characterized by impaired superoxide production, leading to severe infections and granuloma formation.
- Abnormalities in immunoglobulin concentrations have been suggested in CGD, prompting investigation into factors controlling antibody production.
Purpose of the Study:
- To investigate alterations in B-cell activating factor (BAFF) levels and related molecules in patients with Chronic Granulomatous Disease.
- To determine the relationship between BAFF concentrations, inflammation markers, and B-cell receptor expression in CGD.
Main Methods:
- Plasma BAFF concentrations were measured in CGD subjects and healthy donors using quantitative assays.
- BAFF levels were compared between CGD patients with and without IFN-γ treatment.
- In vitro studies assessed BAFF production by CGD leukocytes stimulated with IFN-γ or G-CSF.
- Expression of BAFF receptors (BAFF-R, TACI) on B cells was analyzed.
- Correlations between BAFF, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), IgM, and BAFF response to endotoxin were examined.
Main Results:
- CGD subjects exhibited significantly higher plasma BAFF concentrations (2.3-fold increase) compared to healthy donors (p < 0.0001).
- Patients receiving IFN-γ treatment showed higher BAFF levels than those not on treatment (1.6-fold increase, p < 0.005).
- CGD leukocytes produced normal BAFF levels in vitro upon stimulation.
- B cells from CGD subjects displayed significantly reduced expression of BAFF-R and TACI.
- Elevated BAFF correlated with inflammatory markers (CRP, ESR) and IgM levels in CGD patients.
Conclusions:
- Elevated BAFF in CGD is associated with chronic inflammation and potentially linked to altered B-cell receptor expression.
- These findings suggest that increased BAFF in CGD and healthy individuals may be a response to acute and chronic inflammatory processes.
- BAFF may play a role in the immune dysregulation observed in Chronic Granulomatous Disease.
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