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.
Keywords: B-cells; B-lymphocytes; HMGCR protein; T-cells; T-lymphocytes; autoimmunity; human; immune suppression; inflammatory myopathy.
© 2015 Wiley Periodicals, Inc.