Recurrent
digital ulcers are manifestations of
vascular disease in patients with
systemic sclerosis (SSc). We report six patients with severe
digital ulcers who were treated with
bosentan administered p.o., 62.5-125 mg daily. The mean duration from the diagnosis of SSc to the initiation of
bosentan was 9.5 years, and the observation period after
bosentan administration was from 7 months to 4.5 years. In case 1, neither new
digital ulcers nor Raynaud's phenomenon developed for 4.5 years. In case 2,
digital ulcers recurred after the discontinuation of
bosentan; however, re-administration of
bosentan lead to the improvement. In cases 3-5 with recurrent
digital ulcers, no new lesions have developed. In these five patients,
pain evaluated by visual analog scale was significantly reduced. In three patients,
bosentan was discontinued because of severe
liver dysfunction. These results suggest that
bosentan is an effective treatment for refractory
digital ulcers associated with SSc; however, liver function should be carefully monitored. Compared to the doses of
bosentan used to treat
pulmonary hypertension, relatively lower doses may effectively control painful
digital ulcer/
gangrene in patients with SSc.