Treatment of pulmonary hypertension

Research output: Chapter in Book/Report/Conference proceedingChapter


Pulmonary hypertension (PH) is a significant contributor to the morbidity and mortality associated with systemic sclerosis (SSc). The spectrum of PH in SSc patients includes patients with isolated pulmonary arterial hypertension (SSc-PAH; World Health Organization Group 1) and patients with PH due to pulmonary fibrosis (SSc-ILD-PH; World Health Organization Group 3). In the last 20 years, the combined pulmonary manifestations of SSc have overtaken renal involvement as the primary cause of mortality [1, 2], accounting for up 60% of the SSc-related deaths [2]. Pulmonary arterial hypertension, independent of other pulmonary manifestations, is the second leading cause of mortality in SSc patients [2]. All forms of SSc-related PH share a grim prognosis, and the efficacy of currently available PAH-specific therapies in SSc patients has been disappointing when compared to patients with other forms of PAH.

Original languageEnglish (US)
Title of host publicationScleroderma
Subtitle of host publicationFrom Pathogenesis to Comprehensive Management
PublisherSpringer US
Number of pages9
ISBN (Electronic)9781441957740
ISBN (Print)9781441957733
StatePublished - Jan 1 2012


  • Anti-inflammatory drugs
  • Anticoagulation
  • Calcium channel blockers
  • Efficacy
  • PAH-specific therapies
  • Phosphodiesterase inhibitors
  • Prostaglandins
  • Pulmonary hypertension
  • Treatment

ASJC Scopus subject areas

  • General Medicine


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