Pulmonary veno-occlusive disease and the CREST variant of scleroderma

Can J Cardiol. 1992 Dec;8(10):1055-8.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Female
  • Humans
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / pathology
  • Lung / pathology
  • Middle Aged
  • Pulmonary Veins / pathology
  • Pulmonary Veno-Occlusive Disease / complications*
  • Pulmonary Veno-Occlusive Disease / pathology
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / pathology
  • Syndrome