Signs evocative of venous and capillary (pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement
| Pulmonary function tests | Decreased DLCO (frequently <50%) |
| Severe hypoxaemia | |
| Chest HRCT | Septal lines |
| Centrilobular ground-glass opacities/nodules | |
| Mediastinal lymph node enlargement | |
| Response to PAH therapy | Possible pulmonary oedema |
| Genetic background | Biallelic EIF2AK4 mutations |
| Occupational exposure | Organic solvent (trichloroethylene) |
DLCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography; PAH: pulmonary arterial hypertension.