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Autoimmune necrotising myopathy and HMGCR antibodies
  1. Kushan Karunaratne1,
  2. Dimitri Amiras2,
  3. Matthew C Pickering3,
  4. Monika Hofer4,
  5. Stuart Viegas1
  1. 1Department of Neurology, Imperial College Healthcare NHS Trust, London, UK
  2. 2Department of Radiology, Imperial College Healthcare NHS Trust, London, UK
  3. 3Department of Rheumatology, Imperial College Healthcare NHS Trust, London, UK
  4. 4Department of Neuropathology, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
  1. Correspondence to Dr Stuart Viegas, Neuromuscular Unit, Department of Neurology, Charing Cross Hospital, London W6 8RF, UK; stuart.viegas{at}nhs.net

Abstract

Statins lower serum cholesterol concentrations by inhibiting the enzyme 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR). Muscle side effects are relatively common and include asymptomatic elevation of serum creatine kinase (CK), myalgia, proximal muscle weakness and rhabdomyolysis. More recently, a subset of cases of immune-mediated necrotising myopathy has been found to have antibodies against HMGCR. It is often an aggressive and debilitating myopathy and has a complex pathogenesis characterised by fibre necrosis, usually with minimal associated inflammation. Not all such patients are taking statins. The general consensus is that best treatment involves withdrawing the statin and giving immunosuppressive and immunomodulatory treatment. We describe three cases of HMGCR-related immune-mediated necrotising myopathy, detailing their clinical course and subsequent management, illustrating the spectrum of this disorder.

  • HMG-CoA reductase
  • necrotizing
  • myopathy
  • statins
  • autoantibodies

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Footnotes

  • Contributors SV is the consultant responsible for care of all three patients. MP responsible for care of patient 3. DA and MH have been involved in management of all three patients. All authors have been involved in revising the manuscript.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by David Hilton-Jones, Oxford, UK.

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