Prospects for improving outcomes in systemic sclerosis-related pulmonary hypertension

Internal Medicine Journal
Vivek ThakkarS M Proudman

Abstract

Pulmonary arterial hypertension (PAH) is a leading cause of morbidity and mortality in patients with systemic sclerosis (SSc). Approximately one in 10 will develop PAH during their lifetime. These patients have a worse prognosis than those with PAH due to other causes. The most common clinical feature of SSc-PAH in the early stages is non-specific exercise intolerance that can be erroneously attributed to other manifestations of SSc. Screening provides an opportunity for early identification of SSc-PAH and prompt initiation of therapies with the potential to improve quality of life and survival. International guidelines recommend annual transthoracic Doppler echocardiography (TTE), but TTE has limitations. The tricuspid regurgitant jet required for estimating the systolic pulmonary artery pressure is absent in up to 39% of patients, including a proportion with PAH. This has prompted a move to new screening algorithms that are less dependent on TTE. Not all pulmonary hypertension (PH) in patients with SSc is PAH. Other causes include PH secondary to left heart disease, interstitial lung disease-related PH, chronic thromboembolic PH and pulmonary veno-occlusive disease. With the advent of evidence-based therapies, including newer...Continue Reading

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Citations

May 8, 2016·The American Journal of Medicine·Kavita KhairaEzra A Amsterdam
Jul 17, 2016·Best Practice & Research. Clinical Rheumatology·Vivek Thakkar, Edmund M T Lau
Dec 3, 2016·The European Respiratory Journal·Edmund M T LauPhilippe Herve
Dec 3, 2016·Expert Review of Clinical Immunology·Cosimo BruniMarco Matucci-Cerinic
Jan 20, 2021·Seminars in Arthritis and Rheumatism·Kimberly ShowalterBella Mehta

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