CTICasesPulmonary veno-occlusive disease (PVOD) in mixed connective tissue disease
Imaging findings
CT scan of a 50-year-old male with mixed connective tissue disease and severe pulmonary hypertension shows marked enlargement of the main pulmonary artery (larger than the aorta), right ventricular dilation and hypertrophy, and subtle diffuse ground-glass opacity with bronchial wall thickening. Pathology after lung transplantation confirmed pulmonary veno-occlusive disease (PVOD).
Key takeaways
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension that can complicate mixed connective tissue disease. Radiographic features such as septal thickening, ground-glass nodules, and lymphadenopathy in a patient with clinical PAH should raise suspicion for PVOD.
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