[Bosentan for treatment of active digital ulcers in patients with systemic sclerosis].
OBJECTIVE
To describe the effect of bosentan and its dual inhibition of endothelin-1 ETA and ETB receptors on digital ulcers in patients with systemic sclerosis (SSc).


METHODS
Patients receiving bosentan for SSc-related digital ulcers were identified in eight centers, and their characteristics and follow-up were recorded.


RESULTS
Nine (six with diffuse and three with limited cutaneous forms of SSc) patients (median age: 54 years) had received bosentan for digital ulcers. Complete healing occurred in seven (median time to improvement: 4 weeks). Another experienced a significant decrease in the number of ulcers (from 22 to 5) in 8 weeks, while one had no improvement. After a median follow-up of 24.3 months, only one recurrence was observed. Raynaud phenomenon improved in all but one patient.


CONCLUSIONS
These data suggest that some patients may benefit from bosentan to treat digital ulcers. The short time to healing in these patients with rather chronic ulcers argues strongly in favor of its use. These results also strengthen the evidence that endothelin-1 plays an important role in the vascular manifestations of SSc.


CONCLUSIONS
Bosentan can be effective in the treatment of digital ulcers in some SSc patients with SSc, probably especially those involving substantial ischemia. Bosentan is not a first-line drug in this indication yet and must be carefully used by specialists in SSc. Forthcoming results from the international RAPIDS-2 study should clarify the indications for bosentan in the treatment of SSc-related digital ulcers.
Launay
David
D
Service de Médecine Interne, Centre National de Référence Atteintes Vasculaires de la Sclérodermie, Hôpital Claude-Huriez, CHU de Lille, Université Lille 2. d-launay@chru-lille.fr
Diot
Elisabeth
E
Pasquier
Elisabeth
E
Mouthon
Luc
L
Boullanger
Nadine
N
Fain
Olivier
O
Jego
Patrick
P
Carpentier
Patrick
P
Hatron
Pierre-Yves
PY
Hachulla
Eric
E
D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000276:immunology; D000077300:Bosentan; D019332:Endothelin-1 / Q000037:antagonists & inhibitors*; D005260:Female; D005385:Fingers / Q000098:blood supply* / Q000503:physiopathology; D005500:Follow-Up Studies; D006801:Humans; D007511:Ischemia / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011928:Raynaud Disease / Q000139:chemically induced / Q000453:epidemiology; D012595:Scleroderma, Systemic / Q000188:drug therapy* / Q000453:epidemiology* / Q000276:immunology; D012883:Skin Ulcer / Q000453:epidemiology* / Q000503:physiopathology; D013449:Sulfonamides / Q000009:adverse effects / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome
D000974:Antibodies, Antinuclear; D019332:Endothelin-1; D013449:Sulfonamides; D000077300:Bosentan