Adaptive immune response to therapy in hmgcr autoantibody myopathy.
BACKGROUND
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.
Division of Surgical Sciences, Department of Surgery, Duke University Medical Center, Durham, North Carolina, USA.
John S
JS
Yi
Division of Neuromuscular Diseases, Department of Neurology, Duke University Medical Center, DUMC Box 3403, Durham, North Carolina, 27710, USA.
Melissa A
MA
Russo
Division of Surgical Sciences, Department of Surgery, Duke University Medical Center, Durham, North Carolina, USA.
Kent J
KJ
Weinhold
Division of Neuromuscular Diseases, Department of Neurology, Duke University Medical Center, DUMC Box 3403, Durham, North Carolina, 27710, USA.
Jeffrey T
JT
Guptill
D056704:Adaptive Immunity / Q000502:physiology*; D001323:Autoantibodies / Q000276:immunology*; D006801:Humans; D006903:Hydroxymethylglutaryl CoA Reductases / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009135:Muscular Diseases / Q000276:immunology* / Q000628:therapy*; D009431:Neural Conduction / Q000502:physiology
D001323:Autoantibodies; C553220:HMGCR protein, human; D006903:Hydroxymethylglutaryl CoA Reductases
B-cells; B-lymphocytes; HMGCR protein; T-cells; T-lymphocytes; autoimmunity; human; immune suppression; inflammatory myopathy