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

Clin Rheumatol. 2018 Feb;37(2):543-547. doi: 10.1007/s10067-017-3831-2. Epub 2017 Sep 13.

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.

Keywords: HMG Co-A reductase; Necrotising autoimmune myopathy; Statin; Truncal weakness.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Autoantibodies / blood
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / chemically induced
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / immunology
  • Female
  • Humans
  • Hydroxymethylglutaryl CoA Reductases / immunology
  • Hydroxymethylglutaryl-CoA Reductase Inhibitors / adverse effects*
  • Male
  • Middle Aged
  • Muscle Weakness / blood
  • Muscle Weakness / chemically induced
  • Muscle Weakness / diagnosis*
  • Muscle Weakness / immunology
  • Muscular Diseases / blood
  • Muscular Diseases / chemically induced
  • Muscular Diseases / diagnosis*
  • Muscular Diseases / immunology
  • Retrospective Studies
  • Symptom Assessment

Substances

  • Autoantibodies
  • Hydroxymethylglutaryl-CoA Reductase Inhibitors
  • HMGCR protein, human
  • Hydroxymethylglutaryl CoA Reductases