A case of pulmonary veno-occlusive disease associated with systemic sclerosis.
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.
Saito
Akira
A
Department of Respiratory Medicine, Graduate School of Medicine, University of Tokyo, Japan. asaitou-tky@umin.ac.jp
Takizawa
Hajime
H
Ito
Koji
K
Yamamoto
Kazuhiko
K
Oka
Teruaki
T
D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D006328:Cardiac Catheterization; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy; D014057:Tomography, X-Ray Computed
D000893:Anti-Inflammatory Agents; D011239:Prednisolone