Launay David, Diot Elisabeth, Pasquier Elisabeth, Mouthon Luc, Boullanger Nadine, Fain Olivier, Jego Patrick, Carpentier Patrick, Hatron Pierre-Yves, Hachulla Eric
Service de Médecine Interne, Centre National de Référence Atteintes Vasculaires de la Sclérodermie, Hôpital Claude-Huriez, CHU de Lille, Université Lille 2.
Presse Med. 2006 Apr;35(4 Pt 1):587-92. doi: 10.1016/s0755-4982(06)74645-0.
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).
Patients receiving bosentan for SSc-related digital ulcers were identified in eight centers, and their characteristics and follow-up were recorded.
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.
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.
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.
描述波生坦及其对内皮素-1 ETA和ETB受体的双重抑制作用对系统性硬化症（SSc）患者指端溃疡的影响。
在八个中心确定接受波生坦治疗SSc相关指端溃疡的患者，并记录其特征和随访情况。
9例（6例弥漫性皮肤型和3例局限性皮肤型SSc）患者（中位年龄：54岁）接受波生坦治疗指端溃疡。7例完全愈合（改善的中位时间：4周）。另一例在8周内溃疡数量显著减少（从22个减至5个），而1例无改善。中位随访24.3个月后，仅观察到1例复发。除1例患者外，其余患者雷诺现象均有改善。
这些数据表明，部分患者可能从波生坦治疗指端溃疡中获益。这些患有相当慢性溃疡的患者愈合时间短有力地支持了其使用。这些结果也进一步证明内皮素-1在SSc的血管表现中起重要作用。
波生坦对某些SSc患者的指端溃疡可能有效，可能尤其适用于那些存在明显缺血的患者。波生坦在该适应症中尚未作为一线药物，必须由SSc专科医生谨慎使用。即将公布的国际RAPIDS-2研究结果应能明确波生坦治疗SSc相关指端溃疡的适应症。
