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

Eleni Tiniakou, Erika Rivera, Andrew L. Mammen, Lisa Christopher-Stine

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalArthritis and Rheumatology
DOIs
StateAccepted/In press - Jan 1 2019

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Hydroxymethylglutaryl-CoA Reductase Inhibitors
Muscular Diseases
Muscle Strength
Coenzyme A
Creatine Kinase
Oxidoreductases
Proxy
Hyperlipidemias
Immunosuppression
Proprotein Convertase 9
Anti-Idiotypic Antibodies
Cholesterol
Lipids
Safety
Antibodies
Serum

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology

Cite this

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title = "Use of Proprotein Convertase Subtilisin/Kexin Type 9 Inhibitors in Statin-Associated Immune-Mediated Necrotizing Myopathy: A Case Series",
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.",
author = "Eleni Tiniakou and Erika Rivera and Mammen, {Andrew L.} and Lisa Christopher-Stine",
year = "2019",
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doi = "10.1002/art.40919",
language = "English (US)",
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AB - 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.

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