Systemic sclerosis (SSc) is a connective tissue disease characterized by excessive collagen
deposition, autoimmunity and by vascular hyper-reactivity and obliterative microvascular
phenomena that involves multiple organs. Scleroderma Renal Crisis (SRC) occurs in 5% of
patients and mainly with diffuse cutaneous SSc. The routine use of angiotensin-converting
enzyme inhibitors (ACEI) has been reported to dramatically improve outcome, with a fall of
the 12-month mortality from 76% to less than 15% in the United-States. Despite prognostic
improvement, SRC remains a severe manifestation of SSc and functional outcome and survival
remains poor. Bosentan is a specific, orally active, dual endothelin receptor antagonist that
has recently been approved for the treatment of primary pulmonary arterial hypertension and
for the prevention of ischemic digital ulcers. Bosentan could have therapeutic benefits on
others vascular injuries and particularly in SRC.