Lung biopsy is essential to make this diagnosis. This can be difficult if the<unk>  pressure is high. Investigations Chest X ray may show enlargement of the heart and ill-defined patchy lesions in the lung fields. CT chest typically shows wide spread ill-defined centrilobular nodules of ground glass opacity. This is a nonspecific finding and may be seen in a number of<unk>  diseases. CT<unk>  angiography usually shows enlargement of the main<unk>  artery. When measured by echocardiography or<unk>  angiography, the<unk>  arterial pressure is typically elevated. Differential diagnosis *<unk>  veno-occlusive disease Associations This condition has been reported in patients with Ehlers Danlos syndrome, CREST syndrome and scimitar syndrome.
Pulmonary capillary hemangiomatosis
Pulmonary sclerosing pneumocytoma
Hemangioendothelioma
Pulmonary hyalinizing granuloma
Lymphangiomatosis
Mesothelioma
Multifocal lymphangioendotheliomatosis
Meningioangiomatosis