A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism

E Hachulla, J G Coghlan, E Hachulla, J G Coghlan

Abstract

Evidence suggests that endothelin may have a fundamental role in scleroderma pathogenesis, including pulmonary arterial hypertension (PAH)--a leading cause of death in patients with scleroderma. Development of a new class of drug, endothelin receptor antagonists, heralds an improved outlook for patients with scleroderma and related diseases. Heightened vigilance towards early detection of PAH in scleroderma and a multidisciplinary approach to diagnosis and treatment may improve clinical outcomes for these patients.

Figures

Figure 1
Figure 1
PAH: pathogenesis occurs before clinical symptoms.22 Reproduced, with permission of Elsevier Science from Rich.22

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