TY - JOUR T1 - Is there a role for endothelin-1 receptor antagonists in the treatment of lung fibrosis associated with pulmonary hypertension? JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.01287-2018 VL - 52 IS - 2 SP - 1801287 AU - Jana Grune AU - Wolfgang M. Kuebler Y1 - 2018/08/01 UR - http://erj.ersjournals.com/content/52/2/1801287.abstract N2 - Pulmonary hypertension (PH) is a multi-aetiological haemodynamic and pathophysiological condition defined as an increase in mean pulmonary artery pressure ≥25 mmHg at rest, and classified in five subgroups: 1) pulmonary arterial hypertension (PAH), 2) PH due to left heart disease, 3) PH due to lung disease and/or hypoxia, 4) chronic thromboembolic PH and 5) PH with unclear multifactorial mechanisms [1]. Over the past 20 years, a series of breakthrough therapeutic advances in PAH treatment paved the way for presently four approved drug classes. PAH, however, is a rare disease and the vast majority of PH patients belong to groups 2 and 3 [2], for which no effective therapies have so far been approved [3].It is possible that macitentan has therapeutic effects in patients with advanced IPF and associated PH http://ow.ly/NYMs30loVxO ER -