B-cell activating factor (BAFF) is elevated in chronic granulomatous disease

Clin Immunol. 2013 Aug;148(2):258-64. doi: 10.1016/j.clim.2013.05.007. Epub 2013 May 22.

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.

Keywords: B cell activating factor; B cell receptor; B-cell activating factor; BAFF; BCR; CGD; Chronic Granulomatous Disease; Inflammation; TACI; transmembrane activator and CAML-interactor..

Publication types

  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aging
  • B-Cell Activating Factor / blood
  • B-Cell Activating Factor / genetics
  • B-Cell Activating Factor / metabolism*
  • Biomarkers / blood
  • Case-Control Studies
  • Endotoxins / toxicity
  • Female
  • Granulomatous Disease, Chronic / genetics
  • Granulomatous Disease, Chronic / metabolism*
  • Humans
  • Inflammation
  • Interleukins / blood
  • Leukocytes, Mononuclear
  • Male
  • Middle Aged
  • Tumor Necrosis Factor Ligand Superfamily Member 13 / blood
  • Young Adult

Substances

  • B-Cell Activating Factor
  • Biomarkers
  • Endotoxins
  • Interleukins
  • Tumor Necrosis Factor Ligand Superfamily Member 13
  • interleukin-21