Immune-Mediated Necrotizing Myositis with Hydroxy-3-Methyl-Glutaryl-Coenzyme A Reductase Antibody Positivity in MSA-Negative and Statin-Naive Profile

Statin-naive 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.

Automated summary

Statin-naive immune-mediated necrotizing myopathy (IMNM) is a rare disease with a complicated diagnosis in the absence of a reliable biomarker. This case, the first reported in South India, is unique as it highlights the significance of hydroxy-3-methyl-glutaryl-coenzyme A reductase (HMGCR) antibody in diagnosing IMNM, particularly when myositis-specific antibodies are absent. A comprehensive diagnostic approach, including testing for HMGCR antibody, is recommended for IMNM.