Pulmonary veno-occlusive disease: the bête noire of pulmonary hypertension in connective tissue diseases?

  title={Pulmonary veno-occlusive disease: the b{\^e}te noire of pulmonary hypertension in connective tissue diseases?},
  author={Dermot S. O'Callaghan and Peter Dorfmuller and Xavier Ja{\"i}s and Luc Mouthon and Olivier Sitbon and G{\'e}rald Simonneau and Marc Humbert and David Montani},
  journal={Presse medicale},
  volume={40 1 Pt 2},
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension that may develop in patients with connective tissue diseases (CTD). Most cases have been reported in patients with systemic sclerosis, though associations with systemic lupus erythematosis and mixed connective tissue disease have also been described. PVOD is characterised by progressive obstruction of small pulmonary veins and venules that leads to increased pulmonary vascular resistance, right heart failure and… CONTINUE READING


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