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Scleroderma Pulmonary Veno-Occlusive Disease

Imaging findings

CT chest shows mild subpleural reticulation, severe pulmonary arterial hypertension, and multiple small, centrilobular ground-glass nodules distributed randomly throughout both lungs, with mild septal lines indicating interstitial edema.

Key takeaways

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension. In connective tissue diseases like scleroderma, PVOD/PCH can co-exist, presenting on CT as centrilobular ground-glass nodules and septal lines from post-capillary obstruction.

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