Pulmonary Capillary Hemangiomatosis



Pulmonary Capillary Hemangiomatosis


Jud W. Gurney, MD, FACR





Key Facts


Terminology



  • Rare cause of pulmonary hypertension due to proliferation of alveolar capillaries within lung


Imaging Findings



  • Enlarged pulmonary arteries + centrilobular ground-glass opacities


Top Differential Diagnoses



  • Pulmonary Venoocclusive Disease (PVOD)


  • Primary Pulmonary Hypertension (PPH)


  • Chronic Pulmonary Thromboemboli


Pathology



  • Some evidence that proliferation of thin-walled capillaries in PCH is histologic reaction to PVOD


Clinical Issues



  • Normal pulmonary capillary wedge pressure


  • Prognosis poor: Most patients die within 2 years of diagnosis







Axial NECT shows lobular nodular opacities with ground-glass halos image. Note the enlarged pulmonary artery image and mildly enlarged lymph node image in this patient with pulmonary capillary hemangiomatosis.






Axial NECT shows marked enlargement of the central pulmonary arteries image and faint ground-glass opacities image in pulmonary capillary hemangiomatosis.

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Sep 20, 2016 | Posted by in RESPIRATORY IMAGING | Comments Off on Pulmonary Capillary Hemangiomatosis

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