title: Pulmonary veno-occlusive disease and the CREST variant of scleroderma.
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.
authors.affiliation: Rheumatic Disease Unit, Ottawa General Hospital.
authors.forename: P A
authors.initials: PA
authors.lastname: Morassut
authors.forename: V M
authors.initials: VM
authors.lastname: Walley
authors.forename: C D
authors.initials: CD
authors.lastname: Smith
mesh_terms: D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000473:pathology; D008168:Lung / Q000473:pathology; D008875:Middle Aged; D011667:Pulmonary Veins / Q000473:pathology; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications* / Q000473:pathology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000473:pathology; D013577:Syndrome