
A patient is described with a typical manifestation of pulmonary hypertension associated with limited cutaneous scleroderma, also known as CREST syndrome. The patient was treated with a calcium antagonist, oral anticoagulation and, because of evidence for parenchymal inflammation of the lung, with low-dose prednisone and cyclophosphamide. This treatment resulted in initial improvement of diffusion capacity and exercise tolerance, however, 1 year after diagnosis the patient died of progressive pulmonary hypertension.
CREST Syndrome, Male, Hypertension, Pulmonary, Angiography, Middle Aged, Respiratory Function Tests, Fatal Outcome, Echocardiography, Disease Progression, Humans, Tomography, X-Ray Computed
CREST Syndrome, Male, Hypertension, Pulmonary, Angiography, Middle Aged, Respiratory Function Tests, Fatal Outcome, Echocardiography, Disease Progression, Humans, Tomography, X-Ray Computed
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