Irish Journal of Medical Science (1971 -)

, Volume 185, Issue 4, pp 941–943 | Cite as

Novel antibody associations in immune-mediated necrotising myopathy without inflammation

  • D. Curtin
  • D. Costigan
  • C. McCarthy
  • M. Jansen
  • M. Farrell
  • V. Reid
  • K. O’Rourke
Case Based Review



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.


We describe two cases of immune-mediated necrotizing myopathy each associated with a novel antibody.


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.


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.


Immune mediated Necrotizing myopathy 3-Hydroxy-3-methyl-glutaryl-CoA reductase inhibitor Signal recognition particle Antibody 


Conflict of interest



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Copyright information

© Royal Academy of Medicine in Ireland 2014

Authors and Affiliations

  • D. Curtin
    • 1
  • D. Costigan
    • 2
  • C. McCarthy
    • 3
  • M. Jansen
    • 4
  • M. Farrell
    • 4
  • V. Reid
    • 5
  • K. O’Rourke
    • 1
  1. 1.Dublin Neurological Institute at the Mater Misericordiae University HospitalDublin 7Ireland
  2. 2.Department of NeurologyMater Private HospitalDublinIreland
  3. 3.Department of RheumatologyMater Private HospitalDublinIreland
  4. 4.Department of NeuropathologyBeaumont HospitalDublinIreland
  5. 5.Department of Clinical NeurophysiologyMater Misericordiae University HospitalDublinIreland

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