- idiopathic inflammatory myopathy occur usually in men over 50 years
- gradual muscle weakness (mainly distal)
- weakness finger/wrist flexors out of proportion to finger/wrist extensors and shoulder abductors and weakness of knee extensors disproportionate to the hip flexors (quadriceps and long finger flexors)
- weakness of pharyngeal muscle causes dysphagia in 50%
- mild CK elevation
- muscle biopsy: inflammation and basophilic rimmed vacuoles with diagnostic filamentous inclusions and vacuoles on EM
- IBM is refractory to steroid and immunosuppresive agents
3 comments:
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