Inflammatory myopathies
Dermatomyositis, antisynthetase syndrome, immune-mediated necrotising myopathy and inclusion body myositis. The antibody that predicts each phenotype, the 2017 EULAR/ACR score, ILD and cancer screening, troponin I versus T, and steroids plus an early steroid-sparing drug, IVIg and the anti-MDA5 emergency.
Tier 2Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- Idiopathic inflammatory myopathies (IIM) are a family of autoimmune diseases of muscle, skin and lung. Annual incidence is roughly 1 in 100,000.
- Presentation: subacute symmetrical proximal weakness over weeks to months, often with a rash, ILD, arthritis, Raynaud or dysphagia.
- Myositis-specific antibodies now define the subtypes better than biopsy, and they predict ILD and cancer risk.
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Sources
- Therapeutic Guidelines: Rheumatology (inflammatory myopathies)AU/NZTherapeutic Guidelines Limited (eTG complete), current online version, accessed November 2026
- Therapeutic Guidelines: Dermatology (dermatomyositis)AU/NZTherapeutic Guidelines Limited (eTG complete), current online version, accessed November 2026
- Criteria for the clinical use of immunoglobulin in Australia (inflammatory myopathies)AU/NZNational Blood Authority, current version, accessed November 2026
- Lundberg IE et al. 2017 EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis 2017;76:1955-64EULAR and ACR, 2017 (still current in November 2026; a revision is in development)
- Oldroyd AGS et al. International guideline for idiopathic inflammatory myopathy-associated cancer screening: an IMACS initiative. Nat Rev Rheumatol 2023;19:805-17 (author correction Nat Rev Rheumatol 2024;20:315)International Myositis Assessment and Clinical Studies Group, 2023
- Lilleker JB et al. 272nd ENMC international workshop: 10 Years of progress - revision of the ENMC 2013 diagnostic criteria for inclusion body myositis and clinical trial readiness. Neuromuscul Disord 2024;37:36-51European Neuromuscular Centre, 2024
- Aggarwal R et al. Trial of intravenous immune globulin in dermatomyositis (ProDERM). N Engl J Med 2022;387:1264-78New England Journal of Medicine, 2022
- Vleugels RA et al. A phase 3 trial of brepocitinib in dermatomyositis (VALOR). N Engl J Med 2026;394:1883-93New England Journal of Medicine, 2026
- Brepocitinib (Lisraya), a first orally administered small molecule dual TYK2/JAK1 inhibitor approved for dermatomyositis in adults. Med Chem Res 2026Medicinal Chemistry Research, 2026
- Last updated
- 14 November 2026
What changed
- 14 November 2026 Personal emails, About story and Australian-style copy