Schachna Lionel, Wigley Fredrick M
Division of Rheumatology, Departments of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.
Curr Opin Rheumatol. 2002 Nov;14(6):686-93. doi: 10.1097/00002281-200211000-00010.
Increasing evidence suggests that the vasculopathy of scleroderma is mediated by a number of soluble factors and involves a complex interaction between endothelial cells, smooth muscle cells, extracellular matrix, intravascular coagulation factors, and circulating cells. Novel therapeutic approaches beyond vasodilator therapy are being developed by recognizing important molecular pathways involved in scleroderma vascular disease. The success of this strategy is most evident in pulmonary hypertension, an often fatal complication of scleroderma. In this article, the authors explore therapies for scleroderma that target endothelial cells, smooth muscle cells, reactive oxygen species, and circulating blood cells. The authors highlight clinical trials that have investigated the role of prostacyclin (and its analogues) and bosentan in managing scleroderma-related pulmonary hypertension. Finally, the authors look at the potential role of biomarkers as surrogate indicators of active vascular disease in scleroderma.
越来越多的证据表明，硬皮病的血管病变由多种可溶性因子介导，涉及内皮细胞、平滑肌细胞、细胞外基质、血管内凝血因子和循环细胞之间的复杂相互作用。通过认识到硬皮病血管疾病中重要的分子途径，正在开发血管扩张剂治疗以外的新型治疗方法。这种策略的成功在肺动脉高压中最为明显，肺动脉高压是硬皮病常见的致命并发症。在本文中，作者探讨了针对内皮细胞、平滑肌细胞、活性氧和循环血细胞的硬皮病治疗方法。作者重点介绍了研究前列环素（及其类似物）和波生坦在治疗硬皮病相关肺动脉高压中作用的临床试验。最后，作者探讨了生物标志物作为硬皮病活动性血管疾病替代指标的潜在作用。
