Bosentan treatment of digital ulcers related to autoimmune disorders

Endothelin 1, a powerful endogenous vasoconstrictor and mitogen, may be causal to pulmonary hypertension. Evidence also suggests that endothelin (ET) may have a fundamental role in scleroderma pathogenesis, including pulmonary arterial hypertension (PAH), a leading cause of death in patients with scleroderma. The development of a new class of drug, ET receptor antagonists, provides an improved outlook for patients with scleroderma and related diseases. Increasing vigilance toward early detection of PAH in scleroderma and a multidisciplinary approach to diagnosis and treatment may improve the clinical outcome for these patients. We describe the efficacy and safety of bosentan, an orally active dual ET-receptor antagonist, in patients with digital ulcers. Bosentan increases exercise capacity and improves hemodynamics in patients with pulmonary hypertension, suggesting that ET has an important role in pulmonary hypertension but in our experience also for refractory skin ulcers. Drug Dev Res 72:750755, 2011. (C) 2011 Wiley Periodicals, Inc.