Case Reports
Successful therapy of bosentan-refractory pulmonary arterial hypertension (PAH) with inhalative iloprost
K. Ahmadi-Simab, P. Lamprecht, W.L. Gross
CER2544
2005 Vol.23, N°3
PI 0402, PF 0403
Case Reports
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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.
