Introduction: We evaluated the response to immunosuppression in a case of 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR)-autoantibody myopathy.
Methods: T- and B-cell subsets were determined by flow cytometry pre- and posttherapy.
Results: Baseline immune profiling demonstrated strikingly elevated T-follicular helper (Tfh) cells and plasmablasts. Immunosuppression resulted in clinical improvement and decreased Tfh cells, plasmablasts, and autoantibodies.
Conclusions: Immune profiling in HMGCR-autoantibody myopathy suggests a B-cell-mediated disease. Tfh cells and plasmablasts may be therapeutic biomarkers.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4718836 | PMC |
| http://dx.doi.org/10.1002/mus.24947 | DOI Listing |
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