Skip to main content

Characterization of B cells in muscle-specific kinase antibody myasthenia gravis.

Publication ,  Journal Article
Guptill, JT; Yi, JS; Sanders, DB; Guidon, AC; Juel, VC; Massey, JM; Howard, JF; Scuderi, F; Bartoccioni, E; Evoli, A; Weinhold, KJ
Published in: Neurol Neuroimmunol Neuroinflamm
April 2015

OBJECTIVE: To characterize B-cell subsets in patients with muscle-specific tyrosine kinase (MuSK) myasthenia gravis (MG). METHODS: In accordance with Human Immunology Project Consortium guidelines, we performed polychromatic flow cytometry and ELISA assays in peripheral blood samples from 18 patients with MuSK MG and 9 healthy controls. To complement a B-cell phenotype assay that evaluated maturational subsets, we measured B10 cell percentages, plasma B cell-activating factor (BAFF) levels, and MuSK antibody titers. Immunologic variables were compared with healthy controls and clinical outcome measures. RESULTS: As expected, patients treated with rituximab had high percentages of transitional B cells and plasmablasts and thus were excluded from subsequent analysis. The remaining patients with MuSK MG and controls had similar percentages of total B cells and naïve, memory, isotype-switched, plasmablast, and transitional B-cell subsets. However, patients with MuSK MG had higher BAFF levels and lower percentages of B10 cells. In addition, we observed an increase in MuSK antibody levels with more severe disease. CONCLUSIONS: We found prominent B-cell pathology in the distinct form of MG with MuSK autoantibodies. Increased BAFF levels have been described in other autoimmune diseases, including acetylcholine receptor antibody-positive MG. This finding suggests a role for BAFF in the survival of B cells in MuSK MG, which has important therapeutic implications. B10 cells, a recently described rare regulatory B-cell subset that potently blocks Th1 and Th17 responses, were reduced, which suggests a potential mechanism for the breakdown in immune tolerance in patients with MuSK MG.

Duke Scholars

Altmetric Attention Stats
Dimensions Citation Stats

Published In

Neurol Neuroimmunol Neuroinflamm

DOI

ISSN

2332-7812

Publication Date

April 2015

Volume

2

Issue

2

Start / End Page

e77

Location

United States

Related Subject Headings

  • 3209 Neurosciences
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Guptill, J. T., Yi, J. S., Sanders, D. B., Guidon, A. C., Juel, V. C., Massey, J. M., … Weinhold, K. J. (2015). Characterization of B cells in muscle-specific kinase antibody myasthenia gravis. Neurol Neuroimmunol Neuroinflamm, 2(2), e77. https://doi.org/10.1212/NXI.0000000000000077
Guptill, Jeffrey T., John S. Yi, Donald B. Sanders, Amanda C. Guidon, Vern C. Juel, Janice M. Massey, James F. Howard, et al. “Characterization of B cells in muscle-specific kinase antibody myasthenia gravis.Neurol Neuroimmunol Neuroinflamm 2, no. 2 (April 2015): e77. https://doi.org/10.1212/NXI.0000000000000077.
Guptill JT, Yi JS, Sanders DB, Guidon AC, Juel VC, Massey JM, et al. Characterization of B cells in muscle-specific kinase antibody myasthenia gravis. Neurol Neuroimmunol Neuroinflamm. 2015 Apr;2(2):e77.
Guptill, Jeffrey T., et al. “Characterization of B cells in muscle-specific kinase antibody myasthenia gravis.Neurol Neuroimmunol Neuroinflamm, vol. 2, no. 2, Apr. 2015, p. e77. Pubmed, doi:10.1212/NXI.0000000000000077.
Guptill JT, Yi JS, Sanders DB, Guidon AC, Juel VC, Massey JM, Howard JF, Scuderi F, Bartoccioni E, Evoli A, Weinhold KJ. Characterization of B cells in muscle-specific kinase antibody myasthenia gravis. Neurol Neuroimmunol Neuroinflamm. 2015 Apr;2(2):e77.

Published In

Neurol Neuroimmunol Neuroinflamm

DOI

ISSN

2332-7812

Publication Date

April 2015

Volume

2

Issue

2

Start / End Page

e77

Location

United States

Related Subject Headings

  • 3209 Neurosciences