Hypertension pulmonaire associée à la sarcoïdose

Pulmonary hypertension (PH) is a frequent complication of sarcoidosis. Its mechanisms are multiple and often entangled justifying its belonging to the group 5 of PH. We can distinguish the PH that is proportionate to the parenchymal lesions, the PH resulting from a specific granulomatous vascular disease, and PH due to mechanical compression. Its existence is associated with a poor functional and vital prognosis. Echocardiography is a key consideration in the screening of PH in which the diagnosis must be confirmed by cardiac catheterization. It is recommended to address these patients to a PH competence center for assessment and support. Targeted treatment of PH can only be discussed in severe non-compressive forms. The immunosuppressive treatments may be proposed in the active and compressive types of PH. Their effectiveness in specific granulomatous disease is yet to be evaluated. The eligibility for lung transplantation is to evaluate early in the treatment of young patients with no response to medical treatment.