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

doi:10.1016/j.clim.2013.05.007

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 journal › Article › peer-review

5 Scopus citations

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 language	English (US)
Pages (from-to)	258-264
Number of pages	7
Journal	Clinical Immunology
Volume	148
Issue number	2
DOIs	https://doi.org/10.1016/j.clim.2013.05.007
State	Published - Aug 2013
Externally published	Yes

Keywords

B-cell activating factor
Chronic Granulomatous Disease
Inflammation

ASJC Scopus subject areas

Immunology and Allergy
Immunology

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10.1016/j.clim.2013.05.007

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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

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title = "B-cell activating factor (BAFF) is elevated in Chronic Granulomatous Disease",

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, Chronic Granulomatous Disease, Inflammation",

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

note = "Funding Information: Funding: This research was supported by the Intramural Research Program of the NIH and National Institute of Allergy and Infectious Diseases and the Howard Hughes Medical Institute . ",

year = "2013",

month = aug,

doi = "10.1016/j.clim.2013.05.007",

language = "English (US)",

volume = "148",

pages = "258--264",

journal = "Clinical Immunology",

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AU - Matharu, Kabir

AU - Zarember, Kol A.

AU - Marciano, Beatriz E.

AU - Kuhns, Douglas B.

AU - Spalding, Christine

AU - Garofalo, Mary

AU - Dimaggio, Thomas

AU - Estwick, Tyra

AU - Huang, Chiung Yu

AU - Fink, Danielle

AU - Priel, Debra L.

AU - Fleisher, Thomas A.

AU - Holland, Steven M.

AU - Malech, Harry L.

AU - Gallin, John I.

N1 - Funding Information: Funding: This research was supported by the Intramural Research Program of the NIH and National Institute of Allergy and Infectious Diseases and the Howard Hughes Medical Institute .

PY - 2013/8

Y1 - 2013/8

N2 - 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.

AB - 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.

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

Abstract

Keywords

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