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Adaptive immune response to therapy in hmgcr autoantibody myopathy
Author(s) -
Yi John S.,
Russo Melissa A.,
Weinhold Kent J.,
Guptill Jeffrey T.
Publication year - 2016
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.24947
Subject(s) - autoantibody , inflammatory myopathy , immune system , immunosuppression , myopathy , immunology , medicine , flow cytometry , polymyositis , antibody
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