CTICasesPulmonary Capillary Hemangiomatosis in Scleroderma
Imaging findings
Serial CT scans in a 45-year-old female with scleroderma demonstrate severe right heart strain, characterized by right ventricular hypertrophy and dilation, main pulmonary artery enlargement, and a pulmonary artery catheter in place. The lung parenchyma shows progressive, diffuse, and confluent bilateral ground-glass opacities, with progressive accumulation of pleural effusions and volume overload but without conspicuous interlobular septal thickening.
Key takeaways
Pulmonary capillary hemangiomatosis (PCH) and pulmonary veno-occlusive disease (PVOD) are rare, progressive causes of pulmonary hypertension that are strongly associated with connective tissue diseases like scleroderma. Classically, initiating vasodilators (such as epoprostenol/Flolan) in these patients can cause life-threatening pulmonary edema due to post-capillary obstruction, though some literature suggests subset populations can tolerate and benefit from cautious vasodilator therapy without acute decompensation.
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