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- ACE, angiotensin converting enzyme
- CYC, cyclophosphamide
- ECG, electrocardiography
- FVC, forced vital capacity
- PAH, pulmonary artery hypertension
- PASP, pulmonary artery systolic pressure
- SSC, systemic sclerosis
A 63 year old white woman was admitted to hospital in February 2000 owing to rapidly increasing dyspnoea on exertion (NYHA III), chest pain, and fever. The presence of sclerodactyly with loss of distal parts of the fingers, and megacapillaries at capillaroscopy, allowed the diagnosis of limited cutaneous systemic sclerosis (fig 1). Physical examination also disclosed crackles in the lower lobes of the lungs, systolic murmur with loud pulmonary second sound, and feet oedema.
Laboratory tests included high erythrocyte sedimentation rate (145 mm/1st h), C reactive protein (2530 mg/l, normal <100 mg/l), presence of antinuclear antibodies (1/1280 and speckled pattern on indirect immunofluorescence), mild anaemia and thrombocytopenia (platelet count of 115×109/l), decreased arterial oxygen and carbon dioxide pressures. A chest x ray examination showed cardiomegaly, dilatation of the main pulmonary artery (fig 1), centralisation of pulmonary vascular flow, and reticular opacities in …
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