Pulmonary hypertension in sarcoidosis

Abstract

Pulmonary hypertension (PH) is a relatively common complication of sarcoidosis. In different studies, prevalence of PH in sarcoidosis varies from 6% to 74%. PH associated with sarcoidosis (PHAS) is typically associated with poor prognosis. Though PHAS is diagnosed more often in patients with advanced sarcoidosis, it could occasionally occur without pulmonary parenchymal lesions. Pathophysiology of PHAS is quite complex and includes several mechanisms including pulmonary fibrosis, hypoxic vasoconstriction, pulmonary vascular granulomatous obliteration and/or angiitis, extrinsic pulmonary vascular compression, pulmonary veno-occlusive disease, and left heart disease. Management of PHAS is based on treatment of hypoxemia, active sarcoidosis and comorbidities. A role of systemic steroids in therapy of PHAS remains controversial. Pulmonary arterial hypertension (PAH) specific therapy has not been yet recommended in PHAS, but prostanoids, endothelin receptor antagonists and phosphodiesterase-5 inhibitors were effective in some patients with PHAS in several small trials. Additional well-designed randomized placebo-controlled studies are needed to investigate a role of PAH-specific therapy in certain cohorts of patients with PHAS.