[Treatment of pulmonary hypertension associated with connective tissue disease].

Pulmonary hypertension is the most ominous complication of connective tissue diseases and its treatment has been a big challenge to clinicians. Vasodilators including epoprostenol (prostacyclin), bosentan (endothelin-receptor antagonist), and a sildenafil (phosphodiesterase type 5 inhibitor) have recently become available in Japan. These vasodilators may improve exercise tolerance, hemodynamics, activities of daily life, and the life span. In this article, we review an endothelin-receptor antagonist and phosphodiesterase type 5 inhibitor recently approved as treatment modalities for pulmonary hypertension in Japan.