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A 73 year old woman was admitted to hospital for weight loss, dysphagia with solids, and dyspnoea. She had suffered from Raynaud's phenomenon for 10 years. The main relevant findings are shown in the figures (figs 1-4). Erythrocyte sedimentation rate was normal and C reactive protein was 33 mg/l (normal <5 mg/l). White cell count was normal. Laboratory studies also revealed plasma lactate dehydrogenase of 1362 IU/l (normal 220–480 IU/l) and creatine phosphokinase of 1323 IU/l (normal 15–190 IU/l) consistent with myositis. Myoglobin was 1512 μg/l (normal <70 μg/l). Renal function was normal. Antinuclear antibodies were raised up to 1/2560 with a nucleolar staining pattern and extractable nuclear antigens were negative. Rheumatoid factor was negative. Plain chest radiography and lung function tests were normal. Doppler echocardiography demonstrated normal left ventricular function but severe pulmonary hypertension (pulmonary arterial systolic pressure was 56 mm Hg). Capillaroscopy showed dilated nailfold capillary loops.
- What do the clinical photographs and radiograph show?
- What is the most likely diagnosis?
- What are the possible explanations for bilateral ptosis and myositis?