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Published on: August 26, 2025
Idiopathic pulmonary veno-occlusive disease
Mai Iwaki1, Kazuyoshi Imaizumi, Toyoharu Yokoi
1Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya. mai-i@med.nagoya-u.ac.jp
Internal Medicine (Tokyo, Japan)
|August 5, 2009
Summary
Idiopathic pulmonary veno-occlusive disease (PVOD) is a rare condition causing shortness of breath and severe pulmonary hypertension. Diagnosis requires surgical lung biopsy to confirm characteristic vein occlusions and capillary changes.
Area of Science:
- Cardiology
- Pulmonology
- Pathology
Background:
- Idiopathic pulmonary veno-occlusive disease (PVOD) is a rare and severe cause of pulmonary hypertension.
- Early diagnosis is crucial for management, but often challenging due to non-specific symptoms.
Observation:
- A patient presented with progressive dyspnea and was diagnosed with severe pulmonary hypertension via heart catheterization.
- High-resolution computed tomography (HRCT) revealed diffuse, ill-defined centrilobular ground-glass opacities.
Findings:
- Surgical lung biopsy confirmed idiopathic pulmonary veno-occlusive disease (PVOD).
- Microscopic examination showed widespread occlusion of pulmonary veins and venules.
- Prominent loop-like capillary dilatations were observed, correlating with HRCT findings.
Implications:
- This case highlights the importance of integrating radiological findings with histopathological evidence for diagnosing PVOD.
- Understanding the correlation between HRCT features and pathological findings can aid in earlier detection.
- Further research into the pathogenesis and diagnostic markers of PVOD is warranted.
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