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.