Condition | Potential treatments |
Vascular disease | |
Vasculitis | Glucocorticoids and other anti-inflammatory treatments Pulmonary vasodilators |
Granulomatous vascular involvement | Glucocorticoids and other anti-inflammatory treatments Pulmonary vasodilators |
Veno-occlusive disease | Glucocorticoids and other anti-inflammatory treatments Careful use of pulmonary vasodilators |
Pulmonary embolism (CTEPH) | Anticoagulation Balloon pulmonary angioplasty Pulmonary endarterectomy Pulmonary vasodilators |
Interstitial lung disease | |
Parenchymal lung disease due to granulomas | Glucocorticoids and other anti-inflammatory treatments |
Parenchymal lung disease due to fibrosis | Anti-fibrotic agents |
Hilar and mediastinal distortion | |
Pulmonary artery/vein extrinsic compression | Glucocorticoids and other anti-inflammatory treatments Dilation and/or stenting of compressed vessels |
Fibrosing mediastinitis | Dilation and/or stenting of compressed vessels |
Extrapulmonary disease | |
Left ventricular systolic dysfunction | Glucocorticoids and other anti-inflammatory treatments Diuretics, afterload reduction |
Left ventricular diastolic dysfunction | Diuretics |
Sleep apnoea | CPAP, oxygen and other measures |
Liver disease | Glucocorticoids and other anti-inflammatory treatments |
CTEPH: chronic thromboembolic pulmonary hypertension; CPAP: continuous positive airway pressure.