Immune-mediated necrotizing myositis with hydroxy-3-methyl-glutaryl-coenzyme A reductase antibody positivity in MSA-negative and statin-naive profile

Statin-naïve immune-mediated necrotizing myopathy (IMNM) is a rare disease entity, the diagnosis of which is complicated in the absence of a reliable biomarker. In this context, this case, which is the first one reported from a tertiary care center in South India, is unique. The presence of hydroxy-3-methyl-glutaryl-coenzyme A reductase (HMGCR) antibody supplements the clinical diagnosis, specifically in the serological absence of myositis-specific antibodies. A comprehensive diagnostic approach including HMGCR antibody positivity is advisable in IMNM.