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Therapy of pulmonary arterial hypertension in systemic sclerosis: an update.

Denton, CP; Nihtyanova, SI; (2007) Therapy of pulmonary arterial hypertension in systemic sclerosis: an update. Curr Rheumatol Rep , 9 (2) pp. 158-164.

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Abstract

Pulmonary arterial hypertension (PAH) affects 10% to 15% of patients with systemic sclerosis and is a major cause for disease-related morbidity and mortality. Over the past decade, significant progress has been made in the understanding of the pathophysiologic mechanisms underlying PAH. This progress led to the development of several new treatment options and, as a result, dramatically improved survival among this severely affected cohort. The outcome in patients with scleroderma-related PAH is much worse than that in patients with idiopathic PAH, and unfortunately only a few studies have assessed treatment and outcome among patients suffering from connective tissue disease-related PAH. In recent years, publications of connective tissue disease subgroup analysis from large trials in PAH have emerged. We review the current treatment options for PAH and the evidence for their use in scleroderma-related PAH.

Type: Article
Title: Therapy of pulmonary arterial hypertension in systemic sclerosis: an update.
Location: United States
Keywords: Humans, Hypertension, Pulmonary, Pulmonary Artery, Randomized Controlled Trials as Topic, Scleroderma, Systemic, Treatment Outcome
UCL classification: 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: http://discovery.ucl.ac.uk/id/eprint/738428
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