Anti-HMGCR myopathy is an immune-mediated necrotizing myopathy affecting both statin-exposed and unexposed individuals, with distinct clinical, pathologic, and immunogenetic features.
This review provides a comprehensive overview of Anti-HMGCR myopathy, an immune-mediated necrotizing myopathy often associated with statin exposure, detailing its expanding phenotypic spectrum and management.
Anti-HMGCR myopathy was first recognized and characterized in patients with a history of statin exposure and immune-mediated necrotizing myopathy. After the discovery of anti-HMGCR autoantibodies, several international groups identified and characterized more patients, expanding the phenotypic spectrum of this disease to include pediatric patients and young adults without statin exposure and those with a chronic myopathy resembling limb-girdle muscular dystrophy. We provide a summary of clinical findings, pathologic features, muscle imaging, and immunogenetic risk factors of the disease. We also discuss the current treatment strategies and approaches to monitoring the therapeutic response. Lastly, we briefly summarize the current understanding of the pathophysiology of the disease and postulate a model for autoimmunity initiation and propagation in this disease.
Mohassel et al. (Wed,) conducted a review in Anti-HMGCR Myopathy. Anti-HMGCR myopathy is an immune-mediated necrotizing myopathy affecting both statin-exposed and unexposed individuals, with distinct clinical, pathologic, and immunogenetic features.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: