Plasmablasts as a biomarker for IgG4-related disease, independent of serum IgG4 concentrations

Published on Jan 1, 2015in Annals of the Rheumatic Diseases16.102
· DOI :10.1136/ANNRHEUMDIS-2014-205233
Zachary S. Wallace25
Estimated H-index: 25
(Harvard University),
Hamid Mattoo21
Estimated H-index: 21
(Harvard University)
+ 7 AuthorsJohn H. Stone76
Estimated H-index: 76
(Harvard University)
Sources
Abstract
Objectives We examined the utility of circulating total and IgG4+ plasmablasts as biomarkers of diagnosis and disease activity in IgG4-related disease (IgG4-RD). Materials methods We evaluated patients with active, untreated, biopsy-proven IgG4-RD affecting various organs. Flow cytometry was used to measure total plasmablast and IgG4+ plasmablast counts by gating peripheral blood for CD19lowCD38+CD20−CD27+ cells and CD19lowCD38+CD20−CD27+IgG4+ cells. Serum IgG4 concentrations were measured by nephelometry. We compared 37 IgG4-RD patients to 35 controls, including healthy individuals (n=14) and patients with other inflammatory diseases before treatment (n=21). Results The IgG4-RD patients’ mean age was 59, and 68% were male. Fourteen patients (38%) had three or more organs involved. The IgG4-RD patients had substantially elevated total plasmablast counts (median 4698/mL, range 610–79524/mL) compared to both untreated disease controls (median 592/mL, range 19–4294/mL; p  Conclusions Circulating plasmablasts are elevated in active IgG4-RD, even in patients with normal serum IgG4 concentrations. Plasmablast counts are a potentially useful biomarker for diagnosis, assessing response to treatment, and determining the appropriate time for re-treatment.
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