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Published on: November 18, 2018
Pulmonary veno-occlusive disease and the CREST variant of scleroderma
P A Morassut1, V M Walley, C D Smith
1Rheumatic Disease Unit, Ottawa General Hospital.
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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