Recurrent focal myositis developing into a generalised idiopathic inflammatory myopathy with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase autoantibodies

We present the case of a 43-year-old woman with generalised idiopathic inflammatory myopathy (IIM) with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) autoantibodies (Abs) that developed following recurrent focal myositis. Anti-HMGCR Abs are myositis-specific Abs that are associated with immune-mediated necrotising myopathy, a subtype of IIM that is characterised by relatively prominent and severe muscle involvement, generally necessitating multimodal immunosuppressant treatment. While earlier reports have described patients developing polymyositis following focal myositis, this is the first report to describe a patient developing IIM with anti-HMGCR Abs following focal myositis. Thus, clinicians should be aware of the possibility that focal myositis may develop into a generalised IIM and should instruct the patient and monitor the patient accordingly.

as reported in: BMJ Case Rep. 2019 Nov 2; 12(11):