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

Published by Elsevier Inc.

Bibliographische Detailangaben
Veröffentlicht in:	Clinical immunology (Orlando, Fla.). - 1999. - 148(2013), 2 vom: 17. Aug., Seite 258-64
1. Verfasser:	Matharu, Kabir (VerfasserIn)
Weitere Verfasser:	Zarember, Kol A, Marciano, Beatriz E, Kuhns, Douglas B, Spalding, Christine, Garofalo, Mary, Dimaggio, Thomas, Estwick, Tyra, Huang, Chiung-Yu, Fink, Danielle, Priel, Debra L, Fleisher, Thomas A, Holland, Steven M, Malech, Harry L, Gallin, John I
Format:	Online-Aufsatz
Sprache:	English
Veröffentlicht:	2013
Zugriff auf das übergeordnete Werk:	Clinical immunology (Orlando, Fla.)
Schlagworte:	Journal Article Research Support, N.I.H., Intramural Research Support, Non-U.S. Gov't B cell activating factor B cell receptor B-cell activating factor BAFF BCR CGD Chronic Granulomatous Disease mehr... Inflammation TACI transmembrane activator and CAML-interactor. B-Cell Activating Factor Biomarkers Endotoxins Interleukins Tumor Necrosis Factor Ligand Superfamily Member 13 Interleukin-21 MKM3CA6LT1

Beschreibung
Zusammenfassung:	Published by Elsevier Inc. 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
Beschreibung:	Date Completed 17.09.2013 Date Revised 03.01.2025 published: Print-Electronic Citation Status MEDLINE
ISSN:	1521-7035
DOI:	10.1016/j.clim.2013.05.007