Case history
Case history
A 46-year-old woman presents with a 6-week history of progressive weakness in her thighs and upper arms. She describes difficulty getting out of a chair unaided and complains of fatigue and breathlessness. Over the past 3 months she has noticed swelling of her eyelids and a rash on her face, elbows, and hands. On examination she has a periorbital heliotrope rash, violaceous plaques over the extensor surfaces of both elbows, violaceous papules on the metacarpophalangeal joints, and nail fold capillary dilatation. Proximal muscle strength is symmetrically reduced to 4/5; distal muscle strength is normal. Chest exam reveals fine bilateral basal crepitations.
Other presentations
Extramuscular manifestations may be predominant. Cardiac involvement (e.g., arrhythmias, syncope, or heart failure) is recognized as an important prognostic factor of death. Interstitial lung disease may precede muscular symptoms. Other pulmonary manifestations include drug-induced pneumonitis and pulmonary failure due to thoracic muscle weakness. Systemic features such as fever and weight loss occur especially with coexistent associated connective tissue disease. Other uncommon features are myalgia, arthralgia, dysphagia, Raynaud syndrome, cutaneous calcinosis, and cutaneous necrosis.
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