Therapies for scleroderma-related pulmonary arterial hypertension

Research output: Contribution to journalReview article

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 languageEnglish (US)
Pages (from-to)187-196
Number of pages10
JournalExpert review of respiratory medicine
Volume3
Issue number2
DOIs
StatePublished - Sep 14 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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