Abstract
Summary

Pulmonary hypertension (PH) affects approximately 6% of unselected patients with sarcoidosis but its prevalence is much higher in advanced pulmonary fibrosis. Although destruction of the distal capillary bed and resultant hypoxaemia are central, the underlying mechanisms are multifactorial: left heart dysfunction, specific pulmonary vasculopathy (which can cause pulmonary veno-occlusive disease), local increased vasoreactivity, extrinsic vascular compression and portal hypertension. As a result, some patients exhibit ‘‘disproportionate’’ PH, i.e. more severe than expected from the level of functional impairment. There is no validated screening algorithm for the detection of sarcoidosis-associated PH but recent studies have underlined the role of right heart catheterisation (RHC) to exclude post-capillary PH, which is frequently underestimated by echocardiography. PH is associated with a significantly increased morbidity and mortality in sarcoidosis. The cornerstone of management is supportive therapy and lung transplantation in otherwise eligible patients. Rare cases with nonfibrotic pulmonary disease respond to corticosteroids. Published data on the efficacy and safety of pulmonary arterial hypertension (PAH) agents are scarce and discrepant, requiring further studies.