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Aggressive combination therapy for treatment of systemic sclerosis-associated pulmonary hypertension

Coghlan, JG; Denton, CP; (2018) Aggressive combination therapy for treatment of systemic sclerosis-associated pulmonary hypertension. Journal of Scleroderma and Related Disorders , 3 (1) pp. 30-38. 10.1177/2397198318758422. Green open access

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Abstract

Pulmonary arterial hypertension is an important complication of systemic sclerosis with high mortality but should be regarded as a treatable manifestation of the disease. Management draws on experience from other forms of pulmonary arterial hypertension and benefits from an increasing number of licenced therapies. Outcome is variable but recent clinical trials suggest that combination therapies used early in the disease may be associated with better outcomes. This is important because previous clinical trials using short-term gain in exercise capacity did not show significant benefit compared to that observed for idiopathic or heritable forms of pulmonary arterial hypertension. Thus, it is important to identify cases as early as possible and to manage cases that are in a high-risk group using early combination therapy. This review summarises the most recent analyses of clinical trial data, with a focus on those patients with SSc-associated pulmonary arterial hypertension and provides the evidence base that supports current treatment recommendations for aggressive pulmonary arterial hypertension occurring in systemic sclerosis, including the early use of combination pulmonary arterial hypertension–specific drugs in appropriate cases.

Type: Article
Title: Aggressive combination therapy for treatment of systemic sclerosis-associated pulmonary hypertension
Open access status: An open access version is available from UCL Discovery
DOI: 10.1177/2397198318758422
Publisher version: https://doi.org/10.1177%2F2397198318758422
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Combination therapy, Lung, Pulmonary hypertension, Scleroderma
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/10062764
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