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Updated: Aug 30, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
A case of pulmonary veno-occlusive disease associated with systemic sclerosis
Akira Saito1, Hajime Takizawa, Koji Ito
1Department of Respiratory Medicine, Graduate School of Medicine, University of Tokyo, Japan. asaitou-tky@umin.ac.jp
Abstract:
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.
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