Screening for pulmonary arterial hypertension in systemic sclerosis

European Respiratory Review : an Official Journal of the European Respiratory Society
Jason WeatheraldMarc Humbert

Abstract

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 ...Continue Reading

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Citations

Oct 4, 2019·European Respiratory Review : an Official Journal of the European Respiratory Society·Sergio HarariMarc Humbert
Apr 3, 2020·Respirology : Official Journal of the Asian Pacific Society of Respirology·Jason Weatherald, Marc Humbert
Dec 21, 2019·European Heart Journal Supplements : Journal of the European Society of Cardiology·David G KielyMarc Humbert
Nov 3, 2020·European Journal of Haematology·Alberto FerrariTiziano Barbui
Jan 19, 2020·European Journal of Internal Medicine·Antonio BrucatoCarla Carnovale
Jun 26, 2021·The European Respiratory Journal·David LaunayVincent Sobanski
Aug 20, 2021·European Respiratory Review : an Official Journal of the European Respiratory Society·Ashraful HaqueRobin Condliffe

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