Journal article
Treatment and outcomes in necrotising autoimmune myopathy: an australian perspective
Neuromuscular Disorders, Vol.26(11), pp.734-740
2016
Abstract
Necrotising 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.
Details
- Title
- Treatment and outcomes in necrotising autoimmune myopathy: an australian perspective
- Authors/Creators
- C. Ashton (Author/Creator)R.C. Junckerstorff (Author/Creator) - The University of Western AustraliaC. Bundell (Author/Creator) - Queen Elizabeth II Medical CentreP. Hollingsworth (Author/Creator) - Queen Elizabeth II Medical CentreM. Needham (Author/Creator) - Fiona Stanley Hospital
- Publication Details
- Neuromuscular Disorders, Vol.26(11), pp.734-740
- Publisher
- Elsevier BV
- Identifiers
- 991005543039007891
- Copyright
- © 2016 Elsevier B.V.
- Murdoch Affiliation
- School of Health Professions
- Language
- English
- Resource Type
- Journal article
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- Collaboration types
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- Citation topics
- 1 Clinical & Life Sciences
- 1.106 Rheumatology
- 1.106.1684 Dermatomyositis
- Web Of Science research areas
- Clinical Neurology
- Neurosciences
- ESI research areas
- Neuroscience & Behavior