Background-Few treatments are available for isolated pulmonary hypertension (PHT), which has a high morbidity and mortality. This trial was designed to assess the hemodynamic effects of bosentan, an endothelin receptor antagonist, in patients with PHT, in which local overproduction of endothelin-1 (ET-1) is thought to play a pathogenic role. Methods and Results-An open-label, dose-ranging study was performed in 7 female patients with primary PHT (nϭ5) or isolated PHT associated with limited scleroderma (nϭ2

Inhaled NO is an effective and selective pulmonary vasodilator in a significant number of patients with pulmonary hypertension associated with limited cutaneous systemic sclerosis. It may be useful in determining the potentially reversible contribution to the increased PVR and should be considered for patients with acute pulmonary vascular crisis.

Cuse 2: A 33-year-old Thai female had a long history of SIB dating back to the age of ten years, manifest by arthritis, skin rash, focal proliferative lupus nephritis, cutaneous vasculitis, recurrent thrombocytopenia, Aust NZ J Med 2000; 30 WALLMAN ET AL.

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