TY - JOUR T1 - Pulmonary hypertension in systemic sclerosis: different phenotypes JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW DO - 10.1183/16000617.0056-2017 VL - 26 IS - 145 SP - 170056 AU - David Launay AU - Vincent Sobanski AU - Eric Hachulla AU - Marc Humbert Y1 - 2017/09/30 UR - http://err.ersjournals.com/content/26/145/170056.abstract N2 - Pulmonary hypertension (PH) is a frequent and severe complication of systemic sclerosis (SSc). PH in SSc is highly heterogeneous because of the various clinical phenotypes of SSc itself and because the mechanisms of PH can vary from one patient to another. PH in SSc may be due to vasculopathy of the small pulmonary arteries (group 1; pulmonary arterial hypertension), interstitial lung disease (group 3; PH due to lung disease or chronic hypoxia) or myocardial fibrosis leading to left ventricular systolic or diastolic dysfunction (group 2; PH due to chronic left-heart disease). Pulmonary veno-occlusive disease is not uncommon in SSc and may also cause PH in some patients (group 1′). There is a high prevalence of each of these conditions in SSc and, as such, it may be difficult to determine the dominant cause of PH in a particular patient. However, careful phenotyping of PH in SSc is important as the therapy required for each of these underlying conditions is very different. In this review, we will decipher the different phenotypes of SSc-PH.Pulmonary hypertension in systemic sclerosis is heterogeneous with various possible mechanisms http://ow.ly/s98X30f0lcu ER -