Complete resolution of idiopathic pulmonary arterial hypertension following chemotherapy.

Idiopathic pulmonary arterial hypertension (IPAH) is a rare, chronically progressive disease associated with high mortality. Despite remarkable achievements in treatment, prognosis remains poor with a survival rate of ,60% 3 years after diagnosis [1]. The clinical course of the disease (short survival and no cure), as well as its pathophysiological aspects, reveals analogies between IPAH and neoplastic diseases [2]. Abnormalities of pulmonary vasomotor tone only partly explain the mechanism of IPAH and more irrevocable changes, such as resistance to apoptotic signals of pulmonary arterial endothelial cells, may lead to abnormal angioproliferation and an increase in pulmonary vascular resistance [3].