Treatment of HMGCR Antibody-Associated Myopathy

Initial Management

• The American College of Cardiology recommends immediately ceasing statin therapy if the patient is currently taking one, and patients should never be re-exposed to statins after diagnosis 1
• The American College of Cardiology and Mayo Clinic suggest that statin withdrawal alone will not control the disease, and combination therapy is required to achieve remission, with high-dose corticosteroids initiated immediately upon diagnosis 1, 2

First-Line Immunosuppression

• Mayo Clinic recommends beginning with intravenous methylprednisolone pulse therapy (typically 1000 mg daily for 3-5 days) for severe presentations, and early addition of steroid-sparing agents prevents complications of prolonged high-dose corticosteroid use 2

Critical Pitfalls to Avoid

• Mayo Clinic advises against relying on monotherapy with corticosteroids alone, as nearly all patients require combination therapy to achieve disease control 2