Pearls & Oy-sters: Managing Cholesterol in a Patient With Statin Intolerance Due to Anti-HMG-CoA Reductase–Associated Myopathy

Statins are the first line of treatment for hypercholesterolemia and of prevention of atherosclerotic cardiovascular disease (ASCVD). It is estimated that 1 in 4 Americans over the age of 40 years use statins. In rare cases, patients may develop an autoimmune myopathy associated with antibodies against 3-hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR). Anti-HMGCR-associated myopathy requires immediate discontinuation of statins plus initiation of immunosuppressive therapy. Suspension of statin treatment worsens low-density lipoprotein-cholesterol (LDL-C) control, leading to an increased risk of ASCVD and necessitating commencement of another treatment for dyslipidemia. Unfortunately, the management of dyslipidemia in these patients is still unclear. In this study, we describe the case of a 65-year-old woman with dyslipidemia treated with atorvastatin, who consulted for long-standing muscle pain associated with symmetrical proximal weakness. Laboratory tests showed elevated levels of creatine kinase and anti-HMGCR antibodies. She was diagnosed with an anti-HMGCR-associated myopathy and was successfully treated with corticosteroids and azathioprine as immunosuppressive therapy, followed by ezetimibe for LDL-C reduction. We present key findings for early recognition and treatment of anti-HMGCR-associated myopathy and give recommendations on how to manage hypercholesterolemia in a patient with statin intolerance due to this disease.