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Updated: Apr 21, 2026

Antibody Transfection into Neurons as a Tool to Study Disease Pathogenesis
Published on: September 26, 2012
Novel antibody associations in immune-mediated necrotising myopathy without inflammation
D Curtin1, D Costigan2, C McCarthy3
1Dublin Neurological Institute at the Mater Misericordiae University Hospital, 57 Eccles St., Dublin 7, Ireland. Denis.Curtin@physicians.ie.
Introduction:
The patient presenting with proximal muscle weakness, elevated serum creatinine kinase and myopathic electromyography and biopsy findings has a wide differential diagnosis that includes toxic, autoimmune, paraneoplastic and congenital myopathies. Autoimmune myopathies are important to identify because they may respond to immunosuppressive therapies.
Methods:
We describe two cases of immune-mediated necrotizing myopathy each associated with a novel antibody.
Results:
Case 1 describes a progressive myopathy in a statin user. Antibodies to 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase were identified and the patient responded to steroid therapy. Case 2 describes an aggressive myopathy associated with antibodies to signal recognition particle. There was no response to steroids. Clinical improvement followed treatment with rituximab and cyclophosphamide.
Conclusion:
The identification of myositis-specific antibodies is important because they are associated with distinct clinical phenotypes and may guide the physician in terms of treatment strategies.
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