Signs suggestive of venous and capillary (pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis) involvement
| Pulmonary function tests | Decreased DLCO (<50%). |
| Severe hypoxemia. | |
| High-resolution chest CT | Septal lines, centrilobular ground glass opacities, and mediastinal lymphadenopathy. |
| Response to PAH therapy | Possible pulmonary edema. |
| Genetic background | Biallelic EIF2AK4 mutations. |
| Occupational exposure | Organic solvent (trichloroethylene). |
DLCO, diffusing capacity of the lungs for carbon monoxide; PAH, pulmonary arterial hypertension.