HMGCR Immune-Mediated Necrotizing Myopathy: Review of Clinical Features and Update on Treatment

This AANEM podcast features Dr. Joome Suh, a neurologist and neuromuscular specialist at Brigham and Women's Hospital, interviewed by neuromuscular fellow Dr. Nadia Khalil. They discuss HMG-CoA reductase immune-mediated necrotizing myopathy (HMGCR IMNM), a rare autoimmune muscle disease often associated with statin use. The discussion covers clinical presentation, epidemiologic considerations, histopathologic findings, and treatment approaches, with special focus on Dr. Suh’s recent research published in Muscle and Nerve with co-author Dr. Anthony Amato. Their study found that patients receiving maintenance IVIG as part of their regimen were more likely to have a normal CK at 6 months and the daily prednisone-equivalent was lower. A sub-group analysis suggested IVIG as monotherapy is effective.