Table 4

Signs suggestive of venous and capillary (pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis) involvement

Pulmonary function testsDecreased DLCO (<50%).
Severe hypoxemia.
High-resolution chest CTSeptal lines, centrilobular ground glass opacities, and mediastinal lymphadenopathy.
Response to PAH therapyPossible pulmonary edema.
Genetic backgroundBiallelic EIF2AK4 mutations.
Occupational exposureOrganic solvent (trichloroethylene).
  • DLCO, diffusing capacity of the lungs for carbon monoxide; PAH, pulmonary arterial hypertension.