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

dc.contributor.author Bartoccioni, E
dc.contributor.author Evoli, A
dc.contributor.author Guidon, Amanda C
dc.contributor.author Guptill, JT
dc.contributor.author Howard, JF
dc.contributor.author Juel, Vern Charles
dc.contributor.author Massey, JM
dc.contributor.author Sanders, DB
dc.contributor.author Scuderi, F
dc.contributor.author Weinhold, Kent James
dc.contributor.author Yi, John S
dc.coverage.spatial United States
dc.date.accessioned 2015-06-06T19:17:55Z
dc.date.issued 2015-04
dc.identifier http://www.ncbi.nlm.nih.gov/pubmed/25745635
dc.identifier NEURIMMINFL2014002246
dc.identifier.uri http://hdl.handle.net/10161/10206
dc.description.abstract 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.
dc.language eng
dc.relation.ispartof Neurol Neuroimmunol Neuroinflamm
dc.relation.isversionof 10.1212/NXI.0000000000000077
dc.title Characterization of B cells in muscle-specific kinase antibody myasthenia gravis.
dc.type Journal article
pubs.author-url http://www.ncbi.nlm.nih.gov/pubmed/25745635
pubs.begin-page e77
pubs.issue 2
pubs.organisational-group Basic Science Departments
pubs.organisational-group Clinical Science Departments
pubs.organisational-group Duke
pubs.organisational-group Duke Cancer Institute
pubs.organisational-group Duke Clinical Research Institute
pubs.organisational-group Duke Human Vaccine Institute
pubs.organisational-group Immunology
pubs.organisational-group Institutes and Centers
pubs.organisational-group Neurology
pubs.organisational-group Neurology, Neuromuscular Disease
pubs.organisational-group Pathology
pubs.organisational-group School of Medicine
pubs.organisational-group Surgery
pubs.organisational-group Surgery, Surgical Sciences
pubs.publication-status Published online
pubs.volume 2


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