Steen V, Denton C P, Pope J E, Matucci-Cerinic M
Department of Medicine, Georgetown University Medical Center, Washington, DC 20007, USA.
Rheumatology (Oxford). 2009 Jun;48 Suppl 3:iii19-24. doi: 10.1093/rheumatology/kep105.
RP is an almost universal manifestation of SSc, with 95% of all patients being affected, and resulting in digital ulcers (DUs) in approximately 30% of the patients each year. DUs are a major clinical problem, being associated with substantial morbidity (reduced quality of life, pain, disability and disfigurement) that can escalate to gangrene and amputation. Ideally, the treatment of DUs would improve tissue integrity and viability, promote ulcer healing and reduce the formation of new ulcers. Treatments that have shown potential include calcium channel blockers, prostacyclin analogues and endothelin receptor antagonists. However, until recently, management was based on empirical experience. The recent approval (in Europe) of the dual endothelin receptor antagonist, bosentan, to reduce the number of new DUs in patients with SSc and ongoing DU disease, means that there is now an approved therapy--and new hope--for the treatment of DUs in these severely afflicted patients.
雷诺现象(RP)几乎是系统性硬化症(SSc)的普遍表现,95%的患者都会受到影响,每年约30%的患者会出现指端溃疡(DU)。指端溃疡是一个主要的临床问题,与严重的发病率相关(生活质量下降、疼痛、残疾和毁容),严重时可发展为坏疽和截肢。理想情况下,指端溃疡的治疗应改善组织完整性和活力,促进溃疡愈合并减少新溃疡的形成。已显示出潜力的治疗方法包括钙通道阻滞剂、前列环素类似物和内皮素受体拮抗剂。然而,直到最近,治疗仍基于经验。双内皮素受体拮抗剂波生坦最近(在欧洲)获批用于减少系统性硬化症和持续性指端溃疡疾病患者新指端溃疡的数量,这意味着现在有了一种获批的疗法——以及新的希望——用于治疗这些重症患者的指端溃疡。