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Clinico-serologic features of statin-induced necrotising autoimmune myopathy in a single-centre cohort

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Abstract

Statin-induced necrotising autoimmune myopathy (NAM) is a rare but disabling complication of statin therapy. Data regarding treatment and outcomes in these patients is sparse. We retrospectively identified those patients with a diagnosis of statin-induced NAM who were managed in a single-tertiary referral centre from January 2014 to January 2017. Data regarding clinical features, serology, antibody status and functional outcome was collected. We identified 16 patients diagnosed with statin-induced NAM. Truncal weakness was present in 9/16 patients, of which one patient presented with camptocormia. Following treatment, the mean improvement in the 8-point manual muscle test (MMT8) score was 11 points (range 1–25). Antibodies to 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) were detected in 8/14 patients tested. Of patients who were HMGCR positive, 7/8 had significant truncal weakness, compared with 1/6 who were anti-HMGCR negative. In 4/7 patients who had anti-HMGCR retested following treatment, these antibodies subsequently became undetectable. The disappearance of anti-HMGCR was accompanied by sustained clinical improvement in all four patients. The mean Karnofsky Performance Status (KPS) prior to diagnosis was 89/100, and at latest follow-up had fallen to 68/100. We report a novel association of anti-HMGCR antibodies with truncal weakness in patients with statin-induced NAM. Functional impairments persist despite normalisation of muscle strength. Anti-HMGCR antibodies may disappear with treatment, paralleled by clinical remission of disease. Further prospective clinical trials are needed to determine optimal management strategies for statin-induced NAM.

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Acknowledgements

We would like to acknowledge Dr. Sandy Patel for reviewing and interpreting MRI scans, Dr. Caroline Smith, Dr. Barbara Koszyca and Dr. Sophia Otto for expert histopathological assessment of muscle tissue and Ms. Leah McWilliams for assistance with data collection.

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Authors and Affiliations

  1. Department of Neurology, Royal Adelaide Hospital, Adelaide, South Australia, 5000, Australia

    Michael J. Waters

  2. Department of Rheumatology, Royal Adelaide Hospital, Adelaide, South Australia, 5000, Australia

    Vidya Limaye

  3. Discipline of Medicine, Adelaide University, Adelaide, South Australia, 5000, Australia

    Vidya Limaye

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  1. Michael J. Waters
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Correspondence to Michael J. Waters.

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Waters, M.J., Limaye, V. Clinico-serologic features of statin-induced necrotising autoimmune myopathy in a single-centre cohort. Clin Rheumatol 37, 543–547 (2018). https://doi.org/10.1007/s10067-017-3831-2

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