Use of Proprotein Convertase Subtilisin/Kexin Type 9 Inhibitors in Statin‐Associated Immune‐Mediated Necrotizing Myopathy: A Case Series

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Abstract
Objective: To determine the safety of proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors in patients with statin‐associated anti–3‐hydroxy‐3‐methlyglutaryl coenzyme A reductase (anti‐HMGCR)–positive immune‐mediated necrotizing myopathy (IMNM).Methods: Muscle strength was assessed in anti‐HMGCR–positive patients at each visit before and after initiation of PCSK9 inhibitors. The trends in creatine kinase (CK) levels and serum anti‐HMGCR antibody titers were monitored over time.Results: Among 122 anti‐HMGCR–positive patients, we identified 8 patients who were receiving PCSK9 inhibitors for hyperlipidemia. Patients were followed up for an average of 1.5 years (range 3–37 months), and none exhibited reduction in muscle strength. The mean ± SD CK level prior to the initiation of PCSK9 inhibitors was 956 ± 1,137 IU/liter, which was reduced to 419 ± 393 IU/liter at their last visit. Anti‐HMGCR antibody titers followed a similar trend. Notably, in 2 patients, the initiation of the lipid‐lowering medication was followed by unanticipated spontaneous clinical improvement and reduction in immunosuppression.Conclusion: PCSK9 inhibitors are safe for long‐term use as a cholesterol‐lowering agent in patients with statin‐associated IMNM.
Funding Information
  • Rheumatology Research Foundation

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