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New insights into the recognition, classification and management of systemic sclerosis-associated pulmonary hypertension

Mullin, Christopher J.; Mathai, Stephen C.

Current Opinion in Rheumatology: November 2017 - Volume 29 - Issue 6 - p 561–567
doi: 10.1097/BOR.0000000000000440
RAYNAUD PHENOMENON, SCLERODERMA, OVERLAP SYNDROMES AND OTHER FIBROSING SYNDROMES: Edited by John Varga

Purpose of review Pulmonary hypertension is a common complication of systemic sclerosis (SSc), and remains a leading cause of morbidity and mortality. We will review recent developments in the recognition, classification and treatment of pulmonary hypertension in SSc.

Recent findings Advances in screening for pulmonary arterial hypertension (PAH) and use of exercise haemodynamics may help to identify pulmonary vascular disease earlier in SSc. Recent studies have led to changes in recommendations for adjunct therapy and selection of pulmonary vasodilators for the treatment of SSc-associated PAH.

Summary Recent advances in the diagnosis, classification and management of pulmonary hypertension in SSc have continued to improve our understanding of this challenging disease. Ongoing investigation in the pathogenesis of this disease will afford the opportunity to develop targeted therapies to improve outcomes for SSc patients with pulmonary hypertension.

Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

Correspondence to Stephen C. Mathai, MD, MHS, Johns Hopkins University School of Medicine, 1830 E. Monument St., 5th Floor, Baltimore, MD 21205, USA. Tel: +1 410 614 6311; fax: +1 410 614 7451; e-mail: smathai4@jhmi.edu

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