Successful therapy of bosentan-refractory pulmonary arterial hypertension (PAH) with inhalative iloprost

Clin Exp Rheumatol. 2005 May-Jun;23(3):402-3.

Abstract

Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis. High vascular resistance in PAH arises from an imbalance between vasodilatory mediators (prostacyclin, NO) and vasoconstrictive mediators (endothelin, thromboxane A-2). Inhaled iloprost and the dual endothelin receptor antagonist bosentan have recently been shown to be effective in controlled clinical trials. Our case report demonstrates that patients with bosentan-refractory PAH can be successfully treated with iloprost inhalation.

Publication types

  • Case Reports

MeSH terms

  • Administration, Inhalation
  • Antihypertensive Agents / therapeutic use*
  • Bosentan
  • Exercise Test / drug effects
  • Female
  • Hemodynamics / drug effects
  • Hemodynamics / physiology
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / physiopathology
  • Iloprost / administration & dosage*
  • Middle Aged
  • Pulmonary Artery / pathology
  • Sulfonamides / therapeutic use*
  • Treatment Outcome
  • Vasodilator Agents / administration & dosage*
  • Walking

Substances

  • Antihypertensive Agents
  • Sulfonamides
  • Vasodilator Agents
  • Iloprost
  • Bosentan