Pulmonary veno-occlusive disease and the CREST variant of scleroderma.
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.
Morassut
P A
PA
Rheumatic Disease Unit, Ottawa General Hospital.
Walley
V M
VM
Smith
C D
CD
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