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Myopathies (Polymyositis (PM) (Presentation (Proximal muscle weakness,…
Myopathies
Polymyositis (PM)
Epidemiology
Bimodal: Childhood 5-15 y.o., midlife 30-50 y.o.
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Presentation
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Skin: periungal abnormalities, calcinosis
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Dermatomyositis (DM)
Epidemiology
Bimodal: Childhood 5-15 y.o., midlife 30-50 y.o.
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Presentation
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Skin
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Mechanics Hands
^ prev: arthritis, ILD, fever, Raynaud's
Onset: Acute, severe. Moderate response to therapy.
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2-4x risk cancer in DM (but not in kids): adenocarcinoma, ovarian. DM flare can signal recurrence of cancer.
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IVIg for severe: respiratory muscle weakness, dysphagia/dystonia
If fail steroid/DMARD: IVIg, rituxan, immunosuppressants (e.g. cellcept)
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