In systemic sclerosis patients, Bosentan is safe and effective for digital ulcer prevention and it seems to attenuate the development of pulmonary arterial hypertension

Abstract

Sir, SSc is a chronic connective tissue disease characterized by endothelial dysfunction and fibrosis of the skin and internal organs. Vascular dysfunction is one of the hallmarks of SSc and involves both the macro- and microvasculature. Many of the severe internal organ complications of SSc are vascular, including pulmonary arterial hypertension (PAH) and scleroderma renal crisis [1]. Patients with SSc are at high risk for the development of ischaemic digital ulcers (DUs), which occur in 35–60% of SSc patients. Bosentan treatment reduced the occurrence of new DUs in patients with SSc but had no effect on DU healing. Bosentan was well tolerated and may be a useful adjunct in the management of patients with SSc with recurrent DUs [2]. According to various series, PAH occurs in about 5–9% of SSc patients [3].