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

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.