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Adaptive immune response to therapy in hmgcr autoantibody myopathy
John S Yi1, Melissa A Russo2, Kent J Weinhold1
1Division of Surgical Sciences, Department of Surgery, Duke University Medical Center, Durham, North Carolina, USA.
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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