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inclusion body myositis


common form of inflamatory myopathy
  • most common in the elderly
  • equal sex incidence

  • sporadic idiopathic disease (very rarely familial)
  • misdiagnosed as steroid-resistant polymyositis

symptoms

  • presents as a painless slowly progressive proximal myopathy
  • may cause dysphagia
  • mild to moderate muscle wasting

diagnosis

  • serum creatine kinase levels usually normal or only slightly elevated
  • EMG may show non-specific myopathic features
  • diagnosis on muscle biopsy
  • inclusion bodies seen in rimmed vacuoles in skeletal muscle fibres

treatment

  • steroids and immunosuppression generally ineffective
  • rare patients reported who have made a response to treatment

pathogenesis

  • unknown
  • ubiquitin, prion protein, tau protein found in inclusions
  • abnormal mitchondria seen in some case


James Lowe, DM BM BS MRCPath  -  13 November 1997
Last updated:  1 October 2013


 

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Copyright © 2013, Charles E. Kahn, Jr.