A case of pulmonary veno-occlusive disease associated with systemic sclerosis

Respirology. 2003 Sep;8(3):383-5. doi: 10.1046/j.1440-1843.2003.00451.x.


The case of a patient with pulmonary veno-occlusive disease associated with systemic sclerosis is reported. The patient presented with progressive dyspnoea. Echocardiography and cardiac catheterization study demonstrated right-sided heart failure. The CXR suggested pulmonary hypertension and interstitial pulmonary oedema. We suspected pulmonary veno-occlusive disease based on radiological and haemodynamic findings. Treatment with prednisolone resulted in a reduction in pulmonary arterial pressure and CXR findings improved 2 months later, but no further effect was observed. The patient died 7 months later and at autopsy the lungs showed prominent thickening of the interlobular septa and small branches of pulmonary veins showed intimal thickening.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Anti-Inflammatory Agents / therapeutic use
  • Cardiac Catheterization
  • Diagnosis, Differential
  • Fatal Outcome
  • Humans
  • Male
  • Prednisolone / therapeutic use
  • Pulmonary Veno-Occlusive Disease / diagnosis
  • Pulmonary Veno-Occlusive Disease / drug therapy
  • Pulmonary Veno-Occlusive Disease / etiology*
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / drug therapy
  • Tomography, X-Ray Computed


  • Anti-Inflammatory Agents
  • Prednisolone