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Necrotising Autoimmune Myopathy is a subacute proximal myopathy with high creatine kinase levels and biopsy findings of necrotic and regenerating fibres with minimal inflammation. It is associated with anti-SRP and anti-HMGCR antibodies, malignancy and connective tissue disorders, and is responsive to immunotherapy. This review aims to increase clinician awareness of this rare but potentially treatable condition, by describing the clinical presentation, serological and biopsy findings, and providing an overview of the currently utilised immunotherapy regimens.