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  • Title: Pulmonary veno-occlusive disease and the CREST variant of scleroderma.
    Author: Morassut PA, Walley VM, Smith CD.
    Journal: Can J Cardiol; 1992 Dec; 8(10):1055-8. PubMed ID: 1288837.
    Abstract:
    Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension. The authors describe a 48-year-old female with the Calcinosis-Raynaud's phenomenon-Esophageal dysmotility-Sclerodactyly-Telangiectasia (CREST) variant of scleroderma who developed acute pulmonary hypertension with pulmonary infiltrates and a normal pulmonary capillary wedge pressure. At post mortem examination typical changes of pulmonary veno-occlusive disease were found. Similarities between this and other cases in the literature suggest a possible association between the CREST variant of scleroderma and pulmonary veno-occlusive disease.
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