CTICasesPulmonary Capillary Hemangiomatosis
Imaging findings
Chest CT in a patient with scleroderma and severe pulmonary hypertension shows diffuse, profuse centrilobular ground-glass nodules and interstitial edema. There is associated mediastinal/hilar lymphadenopathy, a moderate pericardial effusion, and a small left atrium.
Key takeaways
Pulmonary capillary hemangiomatosis (PCH) is a rare vascular disorder characterized by the histologically benign proliferation of pulmonary capillaries within alveolar septa. It is closely related to PVOD, and both lead to post-capillary pulmonary hypertension. On CT, PCH is characterized by profuse centrilobular ground-glass nodules. Pericardial effusions are common in connective tissue disease-associated pulmonary hypertension and carry a poor prognosis.
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