PT - JOURNAL ARTICLE AU - Weatherald, Jason AU - Montani, David AU - Jevnikar, Mitja AU - Jaïs, Xavier AU - Savale, Laurent AU - Humbert, Marc TI - Screening for pulmonary arterial hypertension in systemic sclerosis AID - 10.1183/16000617.0023-2019 DP - 2019 Sep 30 TA - European Respiratory Review PG - 190023 VI - 28 IP - 153 4099 - http://err.ersjournals.com/content/28/153/190023.short 4100 - http://err.ersjournals.com/content/28/153/190023.full SO - EUROPEAN RESPIRATORY REVIEW2019 Sep 30; 28 AB - Pulmonary arterial hypertension (PAH) is a dreaded complication of systemic sclerosis (SSc) that occurs in ∼10% of patients. Most individuals present with severe symptoms, significant functional impairment and severe haemodynamics at diagnosis, and survival after PAH diagnosis is poor. Therefore, early diagnosis through systematic screening of asymptomatic patients has the potential to identify PAH at an early stage. Current evidence suggests that early diagnosis and treatment of PAH in patients with SSc may lead to better clinical outcomes. Annual screening may include echocardiography, but this can miss some patients due to suboptimal visualisation or insufficient tricuspid regurgitation. Other options for screening include the DETECT algorithm or the use of a combination of pulmonary function testing (forced vital capacity/diffusing capacity of the lung for carbon monoxide ratio) and N-terminal-pro-brain natriuretic peptide levels. Symptomatic patients, those with an elevated tricuspid regurgitation velocity on echocardiogram with or without secondary echocardiographic features of PAH, and those who screen positive on the DETECT or other pulmonary function test algorithms should undergo right heart catheterisation. Exercise echocardiography or cardiopulmonary exercise testing, nailfold capillaroscopy and molecular biomarkers are promising but, as yet, unproven potential options. Future screening studies should employ systematic catheterisation to define the true predictive values for PAH.Screening can detect PAH at an early stage of the disease, which permits earlier medical interventions and may improve outcomes in systemic sclerosis patients. bit.ly/2Q5akGu