MRCEM Notes

Polymyositis

Overview
  • inflammatory disorder causing symmetrical, proximal muscle weakness
  • thought to be a T-cellmediated cytotoxic process directed against muscle fibres
  • may be idiopathic or associated with connective tissue disorders
  • associated with malignancy
  • dermatomyositis is a variant of the disease where skin manifestations are prominent, for example a purple (heliotrope) rash on the cheeks and eyelids
  • typically affects middle-aged, female:male 3:1

Features
  • proximal muscle weakness +/- tenderness
  • Raynaud's
  • respiratory muscle weakness
  • interstitial lung disease: e.g. fibrosing alveolitis or organising pneumonia
  • dysphagia, dysphonia

Investigations
  • elevated creatine kinase
  • EMG
  • muscle biopsy
  • anti-Jo-1 antibodies are seen in pattern of disease associated with lung involvement, Raynaud's and fever
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