Dermatomyositis and polymyositis are inflammatory myositis
Polymyositis presentation: proximal myopathy, sob
Dermatomyositis presentation: rash- sun exposed, violet + proximal myopathy
Associated with ILD
Polymyositis and dermatomyositis are where the immune system attacks the patients muscles
Two peaks of onset: 40yrs and 60yrs
Associations: pulmonary fibrosis; malignancy of lung, breast, female genital tract, stomach & rectum
History:
3 month: progressive muscle weakness- shoulders & hips
40-60F
SoB, dysphagia
Examination:
Proximal lung weakness
Rash- violet, sun exposure distribution, heliotrope (dermatomyositis)
Bilateral inspiratory crackles
Investigations
Raised ESR & CRP
CK raised significantly
Anti Jo: specificity 20% (polymyositis)
Anti-Mi (highly specific, 25% sensitive)
Muscle biopsy
EMG
Polymyositis Diagnostic criteria: symptoms without rash + biopsy
Dermatomyositis diagnostic criteria: symptoms with rash + biopsy
Electromyography and raised CK helpful
Anti Jo antibodies not useful as not sensitive or specific
Differentials: Muscular dystrophy
Acute
Prednisolone (acute)
Azathioprine (steroid sparing, LT)
Cyclophosphamide or cyclosporin if evidence of lung disease
Rituxumab
Created in 2024.