Treatment and outcomes in necrotising autoimmune myopathy: An Australian perspective

C. Ashton, Reimar Junckerstorff, C. Bundell, P. Hollingsworth, M. Needham

    Research output: Contribution to journalArticle

    10 Citations (Scopus)

    Abstract

    © 2016Necrotising Autoimmune Myopathy (NAM) presents as a subacute proximal myopathy with high creatine kinase levels. It is associated with statin exposure, 3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody, connective tissue diseases, signal recognition particle (SRP) antibody and malignancy. This case series presents our Western Australian NAM patient cohort: comparing the subgroup presentations, biopsy appearance and treatment outcomes. We retrospectively collected data on patients diagnosed with NAM at the Western Australian Neuroscience Research Institute between the years 2000 and 2015. We identified 20 patients with Necrotising Autoimmune Myopathy: 14 with anti-HMGCR antibodies; two with anti-SRP antibodies; three with connective tissue disease; two as yet unspecified. Median creatine kinase level was 6047units/L (range 1000–17000). The statin naïve patients with HMGCR antibodies and patients with SRP antibodies were the most severely affected subgroups, with higher creatine kinase levels, and were more resistant to immunotherapy. Two or more immunotherapy agents were required in 90%; eight patients required IVIG and rituximab. Steroid weaning commonly precipitated relapses. Four patients had complete remission, and the remaining patients still require immunotherapy. Necrotising Autoimmune Myopathy is a potentially treatable myopathy, which can be precipitated by statin therapy and requires early, aggressive immunotherapy, usually requiring multiple steroid sparing agents for successful steroid weaning.
    Original languageEnglish
    Pages (from-to)734-740
    JournalNeuromuscular Disorders
    Volume26
    Issue number11
    DOIs
    Publication statusPublished - 2016

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    Muscular Diseases
    Signal Recognition Particle
    Hydroxymethylglutaryl CoA Reductases
    Hydroxymethylglutaryl-CoA Reductase Inhibitors
    Immunotherapy
    Antibodies
    Creatine Kinase
    Connective Tissue Diseases
    Steroids
    Weaning
    Intravenous Immunoglobulins
    Neurosciences
    Biopsy
    Recurrence

    Cite this

    Ashton, C. ; Junckerstorff, Reimar ; Bundell, C. ; Hollingsworth, P. ; Needham, M. / Treatment and outcomes in necrotising autoimmune myopathy: An Australian perspective. In: Neuromuscular Disorders. 2016 ; Vol. 26, No. 11. pp. 734-740.
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    abstract = "{\circledC} 2016Necrotising Autoimmune Myopathy (NAM) presents as a subacute proximal myopathy with high creatine kinase levels. It is associated with statin exposure, 3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody, connective tissue diseases, signal recognition particle (SRP) antibody and malignancy. This case series presents our Western Australian NAM patient cohort: comparing the subgroup presentations, biopsy appearance and treatment outcomes. We retrospectively collected data on patients diagnosed with NAM at the Western Australian Neuroscience Research Institute between the years 2000 and 2015. We identified 20 patients with Necrotising Autoimmune Myopathy: 14 with anti-HMGCR antibodies; two with anti-SRP antibodies; three with connective tissue disease; two as yet unspecified. Median creatine kinase level was 6047units/L (range 1000–17000). The statin na{\"i}ve patients with HMGCR antibodies and patients with SRP antibodies were the most severely affected subgroups, with higher creatine kinase levels, and were more resistant to immunotherapy. Two or more immunotherapy agents were required in 90{\%}; eight patients required IVIG and rituximab. Steroid weaning commonly precipitated relapses. Four patients had complete remission, and the remaining patients still require immunotherapy. Necrotising Autoimmune Myopathy is a potentially treatable myopathy, which can be precipitated by statin therapy and requires early, aggressive immunotherapy, usually requiring multiple steroid sparing agents for successful steroid weaning.",
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    Treatment and outcomes in necrotising autoimmune myopathy: An Australian perspective. / Ashton, C.; Junckerstorff, Reimar; Bundell, C.; Hollingsworth, P.; Needham, M.

    In: Neuromuscular Disorders, Vol. 26, No. 11, 2016, p. 734-740.

    Research output: Contribution to journalArticle

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