B-cell activating factor (BAFF) is elevated in Chronic Granulomatous Disease

Kabir Matharu, Kol A. Zarember, Beatriz E. Marciano, Douglas B. Kuhns, Christine Spalding, Mary Garofalo, Thomas Dimaggio, Tyra Estwick, Chiung Yu Huang, Danielle Fink, Debra L. Priel, Thomas A. Fleisher, Steven M. Holland, Harry L. Malech, John I. Gallin

Research output: Contribution to journalArticle

Abstract

Chronic Granulomatous Disease (CGD) is an inherited defect in superoxide production leading to life-threatening infections, granulomas, and, possibly, abnormal immunoglobulin concentrations. We investigated whether factors controlling antibody production, such as B-cell activating factor (BAFF), were altered in CGD. CGD subjects had significantly increased mean (2.3-fold, p. <. 0.0001) plasma concentrations of BAFF compared to healthy donors. Patients on IFN-γ treatment had significantly higher BAFF concentrations compared with CGD patients not taking IFN-γ (1.6-fold, p. <. 0.005). Leukocytes from CGD subjects produced normal amounts of BAFF in response to IFN-γ or G-CSF in vitro. Expression of BAFF-R and TACI was significantly reduced on CGD B cells. Elevated BAFF in CGD correlated with CRP ( R= 0.44), ESR ( R= 0.49), and IgM ( R= 0.47) and increased rapidly in healthy subjects following intravenous endotoxin administration. These findings suggest that elevated BAFF in CGD subjects and healthy donors is a consequence of acute and chronic inflammation.

Original languageEnglish (US)
Pages (from-to)258-264
Number of pages7
JournalClinical Immunology
Volume148
Issue number2
DOIs
StatePublished - Aug 1 2013

Keywords

  • B-cell activating factor
  • Chronic Granulomatous Disease
  • Inflammation

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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    Matharu, K., Zarember, K. A., Marciano, B. E., Kuhns, D. B., Spalding, C., Garofalo, M., Dimaggio, T., Estwick, T., Huang, C. Y., Fink, D., Priel, D. L., Fleisher, T. A., Holland, S. M., Malech, H. L., & Gallin, J. I. (2013). B-cell activating factor (BAFF) is elevated in Chronic Granulomatous Disease. Clinical Immunology, 148(2), 258-264. https://doi.org/10.1016/j.clim.2013.05.007