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Atypical presentation of anti-HMGCR myopathy
  1. Rahul Karna1,
  2. Richa Singh1,
  3. Cody Marshall2 and
  4. Alexandra Johnston1
  1. 1Internal Medicine, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA
  2. 2Pathology, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA
  1. Correspondence to Dr Rahul Karna; rahulkarnagovind{at}


Immune-mediated necrotising myopathy is a subtype of idiopathic inflammatory myopathy characterised by muscle fibre necrosis without significant inflammatory infiltrate. Anti-3-hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR) myopathy is seen in 6%–10% of idiopathic inflammatory myopathy and is diagnosed in the context of elevated serum creatine kinase levels, proximal muscle weakness and anti-HMGCR autoantibodies. We recently encountered a 61-year-old man with anti-HMGCR myopathy with an atypical skin manifestation, partially responsive to triple therapy with steroids, intravenous immunoglobulin (IVIG) and rituximab. To our knowledge, there have been only four reported cases of skin rash associated with anti-HMGCR myopathy. Our case demonstrates the importance of recognising atypical manifestations of anti-HMGCR myopathy. Early addition of IVIG and rituximab is also critical in patients not responding to steroid monotherapy. Delay in achieving remission leads to prolonged steroid use, lower likelihood of beginning physical therapy and overall worse clinical outcomes.

  • muscle disease
  • rheumatology

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  • Twitter @RahulKarnaMD, @AlexJohnstonDO

  • Contributors RK is the main author of this case report. RK did the groundwork, reviewing articles for referencing and discussion. RK wrote the initial draft, formatted and reviewed final version. RK also coordinated with the patient and was the main person that communicated with the patient. RS helped extract patient’s data from electronic medical records. She also identified suitable articles to be included in the discussion. CM contributed by reviewing pathology slides, editing, formatting and reviewing of the case report. AJ acted as the supervisor for this case report. She helped with the final draft and edits.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.