Adaptive immune response to therapy in hmgcr autoantibody myopathy

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Abstract

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. Muscle Nerve 53: 313–317, 2016

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