Therapy for pulmonary arterial hypertension associated with systemic sclerosis

Curr Opin Rheumatol. 2009 Nov;21(6):642-8. doi: 10.1097/BOR.0b013e3283307dc8.


Purpose of review: Systemic sclerosis is commonly complicated by pulmonary arterial hypertension (PAH-SSc) and is a leading cause of death in this population. We will review existing challenges and recent advances in the treatment of this disease.

Recent findings: Traditionally employed outcome measures in pulmonary arterial hypertension research may not be applicable in PAH-SSc. Importantly, new therapies that target abnormal cellular proliferation in the pulmonary vasculature are currently under investigation and may be particularly relevant to PAH-SSc.

Summary: Pulmonary arterial hypertension complicating systemic sclerosis occurs commonly and portends a poor prognosis. However, recent advances in our understanding of the disease in the context of systemic sclerosis may lead to novel diagnostic and therapeutic strategies that will ultimately improve quality of life and survival in this population.

Publication types

  • Review

MeSH terms

  • Algorithms
  • Anticoagulants / therapeutic use
  • Calcium Channel Blockers / therapeutic use
  • Drug Therapy, Combination
  • Endothelin Receptor Antagonists
  • Epoprostenol / therapeutic use
  • Evidence-Based Medicine
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / therapy*
  • Lung Transplantation
  • Phosphodiesterase Inhibitors / therapeutic use
  • Scleroderma, Systemic / complications*


  • Anticoagulants
  • Calcium Channel Blockers
  • Endothelin Receptor Antagonists
  • Phosphodiesterase Inhibitors
  • Epoprostenol