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

Authors: Jain, Manu¹; Varga, John²

Source: Expert Opinion on Pharmacotherapy, Volume 7, Number 11, August 2006 , pp. 1487-1501(15)

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Abstract:

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.

Document Type: Drug Evaluation

DOI: http://dx.doi.org/10.1517/14656566.7.11.1487

Affiliations: 1: 1Division of Pulmonary and Critical Care, Feinberg School of Medicine, Northwestern University, 240 E. Huron Avenue, M-321, IL, USA., Email: m-jain@northwestern.edu 2: 2Division of Rheumatology, Feinberg School of Medicine, Northwestern University, IL, USA

Publication date: 2006-08-01

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