Successful treatment of a patient with mitochondrial myopathy with alirocumab.

A 48-year-old man presented to our lipid clinic with statin intolerance and elevated serum creatine kinase levels, being affected by mitochondrial myopathy because of heteroplasmic mitochondrial DNA missense mutation in MTCO1 gene (m.7671T>A). He had just been treated with a coronary artery bypass 4 years before because of acute coronary syndrome, and he had consistently high levels of both low-density lipoprotein cholesterol and triglycerides. Dyslipidemia was successfully treated using 75 mg of alirocumab subcutaneously every 2 weeks, 10 mg of ezetimibe daily, 2 g of marine omega-3 fatty acids daily, and 145 mg of micronized fenofibrate every 2 days. Although muscle weakness persisted, myalgia did not reoccur and serum creatine kinase levels remained almost stable over the time. • Lipid-lowering management of patients with mitochondrial myopathy is challenging. • In patients with inherited myopathies, statins have to be used with caution. • Treatment with proprotein convertase subtilisin/kexin type 9 inhibitors does not interact with muscle metabolism. • Proprotein convertase subtilisin/kexin type 9 inhibitors can be safely used in patients with mitochondrial myopathy. [ABSTRACT FROM AUTHOR]