Therapies for scleroderma-related pulmonary arterial hypertension

Paul M. Hassoun

doi:10.1586/ers.09.5

Therapies for scleroderma-related pulmonary arterial hypertension

Paul M. Hassoun

School of Medicine

Research output: Contribution to journal › Review article › peer-review

19 Scopus citations

Abstract

Pulmonary arterial hypertension (PAH), a common complication of systemic sclerosis, carries a very severe prognosis and is one of the leading causes of death in patients who suffer from it. Indeed, response to modern medical therapy has been disappointing in scleroderma-related PAH compared with other forms of PAH from the WHO group 1 classification of diseases, despite similar histological changes involving the pulmonary vasculature. This review discusses specific features of scleroderma-related PAH, currently available and US FDA-approved therapy for this syndrome, as well as potential future therapeutic developments based on newly acquired knowledge of this disorder.

Original language	English (US)
Pages (from-to)	187-196
Number of pages	10
Journal	Expert review of respiratory medicine
Volume	3
Issue number	2
DOIs	https://doi.org/10.1586/ers.09.5
State	Published - 2009

Keywords

Endothelin receptor antagonist
Phosphodiesterase inhibitor
Prostaglandin
Pulmonary arterial hypertension
Scleroderma
Therapy

ASJC Scopus subject areas

Immunology and Allergy
Pulmonary and Respiratory Medicine
Public Health, Environmental and Occupational Health

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10.1586/ers.09.5

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title = "Therapies for scleroderma-related pulmonary arterial hypertension",

abstract = "Pulmonary arterial hypertension (PAH), a common complication of systemic sclerosis, carries a very severe prognosis and is one of the leading causes of death in patients who suffer from it. Indeed, response to modern medical therapy has been disappointing in scleroderma-related PAH compared with other forms of PAH from the WHO group 1 classification of diseases, despite similar histological changes involving the pulmonary vasculature. This review discusses specific features of scleroderma-related PAH, currently available and US FDA-approved therapy for this syndrome, as well as potential future therapeutic developments based on newly acquired knowledge of this disorder.",

keywords = "Endothelin receptor antagonist, Phosphodiesterase inhibitor, Prostaglandin, Pulmonary arterial hypertension, Scleroderma, Therapy",

author = "Hassoun, {Paul M.}",

note = "Funding Information: Paul Hassoun{\textquoteright}s research is supported by NHLBI grant HL084946. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.",

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doi = "10.1586/ers.09.5",

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N1 - Funding Information: Paul Hassoun’s research is supported by NHLBI grant HL084946. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

PY - 2009

Y1 - 2009

N2 - Pulmonary arterial hypertension (PAH), a common complication of systemic sclerosis, carries a very severe prognosis and is one of the leading causes of death in patients who suffer from it. Indeed, response to modern medical therapy has been disappointing in scleroderma-related PAH compared with other forms of PAH from the WHO group 1 classification of diseases, despite similar histological changes involving the pulmonary vasculature. This review discusses specific features of scleroderma-related PAH, currently available and US FDA-approved therapy for this syndrome, as well as potential future therapeutic developments based on newly acquired knowledge of this disorder.

AB - Pulmonary arterial hypertension (PAH), a common complication of systemic sclerosis, carries a very severe prognosis and is one of the leading causes of death in patients who suffer from it. Indeed, response to modern medical therapy has been disappointing in scleroderma-related PAH compared with other forms of PAH from the WHO group 1 classification of diseases, despite similar histological changes involving the pulmonary vasculature. This review discusses specific features of scleroderma-related PAH, currently available and US FDA-approved therapy for this syndrome, as well as potential future therapeutic developments based on newly acquired knowledge of this disorder.

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KW - Phosphodiesterase inhibitor

KW - Prostaglandin

KW - Pulmonary arterial hypertension

KW - Scleroderma

KW - Therapy

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Therapies for scleroderma-related pulmonary arterial hypertension

Abstract

Keywords

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