Bosentan for the treatment of systemic sclerosis-associated pulmonary arterial hypertension, pulmonary fibrosis and digital ulcers.

摘要

Systemic sclerosis (SSc) is a devastating multisytemic autoimmune disease associated with widespread vascular damage. Pulmonary arterial hypertension (PAH) occurs in a significant proportion of patients and contributes to the morbidity and mortality that occurs in this disease. The recent development of specific therapies for the treatment of PAH mandates the early recognition, appropriate evaluation and judicious management of PAH in patients with SSc. Because endothelin (ET)-1 plays an important role in the development of PAH in SSc, and may also contribute to the vascular damage and fibrosis that occur in multiple organs in patients with the disease, inhibiting the production and activity of ET-1 is an appealing strategy for the treatment of SSc. This article reviews the pathophysiology of SSc and its vascular complications, and critically evaluates the current knowledge regarding the potential role of the ET-1 receptor blocker bosentan in the management of patients with SSc.