Adaptive immune response to therapy in hmgcr autoantibody myopathy.
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.
Yi, JS; Russo, MA; Weinhold, KJ; Guptill, JT
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