From Serendipity to Science: How Anti-HMGCR Antibodies Changed Our Understanding of Myositis - PubMed
2 days ago
- #anti-HMGCR
- #statin-induced myositis
- #necrotizing myopathy
- The anti-HMGCR antibody was identified in patients with necrotizing myopathy linked to statin exposure, providing a specific biomarker and mechanistic link to the drug.
- Pathogenesis involves statins upregulating HMGCR in susceptible individuals, with antibodies causing necrosis via complement activation and disrupting HMGCR function, leading to lipid accumulation.
- The disease can occur in statin-naïve patients, including children, mimicking muscular dystrophy, with HLA-DRB1*11:01 as a strong risk allele and higher risk in certain Indigenous populations.
- Anti-HMGCR myopathy persists years after statin exposure and responds to immunotherapy, with intravenous Ig and rituximab as key treatments, while complement-targeting trials showed negative results.