Figure 3.

Proposed therapeutic approach of SAPH based on the mechanisms and phenotypes involved. * The therapeutic approach should be multidisciplinary, involving a sarcoidosis and a PH expert, and take into account the mechanisms involved in the development of PH, the severity of PH and the severity of the underlying parenchymal lung disease. ^† Anti-inflammatory treatment can be initiated before PAH-targeted therapy or in parallel. ^¶ Referral for lung transplantation should not be delayed. Abbreviations: SAPH: sarcoidosis-associated pulmonary hypertension, CTA: computed tomography angiography, V/Q: ventilation/perfusion, AC: anticoagulant, CTEPH: chronic thromboembolic pulmonary hypertension, FVC: forced vital capacity, 18FDG-PET: 18F-2-fluoro-2-deoxy-D-glucose positron emission tomography, PVR: pulmonary vascular resistance, PAH: pulmonary arterial hypertension.