Rheumatology Unit, Department of Internal Medicine, University of Modena and Reggio Emilia, Modena, Italy.
Clin Exp Dermatol. 2010 Dec;35(8):885-7. doi: 10.1111/j.1365-2230.2010.03847.x.
Systemic sclerosis (SSc) is often complicated by severe skin ulcers that are unresponsive to traditional treatments. Vascular alterations are responsible for the ischaemic features of the disease in both the skin and visceral organs. Defective neoangiogenesis correlates with an abnormally reduced quantity of circulating endothelial progenitor cells (EPCs) caused by impaired maturation potential and proliferative capacity of bonemarrow endothelial stem cells. We report a patient with nonhealing cutaneous ulcers successfully treated with recombinant human erythropoietin (rHuEPO). The possible biological effects of this drug were also investigated. Before rHuEPO treatment, the bone-marrow sample contained reduced numbers of EPCs, which were functionally impaired. After a 6-month rHuEPO cycle, a marked increase in endothelial progenitor markers was seen, along with a significant reduction in their apoptotic rates. The clinical and laboratory data variations before and after rHuEPO treatment give new insights into the pathogenetic role of impaired endothelial stem-cell maturation and defective neoangiogenesis in patients with SSc.
系统性硬化症（SSc）常伴有严重的皮肤溃疡，对传统治疗方法无反应。血管改变是皮肤和内脏器官疾病缺血特征的原因。新生血管形成缺陷与循环内皮祖细胞（EPC）的异常减少有关，这是由于骨髓内皮干细胞的成熟潜能和增殖能力受损所致。我们报告了一例用重组人促红细胞生成素（rHuEPO）成功治疗的难治性皮肤溃疡患者。还研究了这种药物的可能生物学效应。在 rHuEPO 治疗前，骨髓样本中 EPC 的数量减少，且功能受损。经过 6 个月的 rHuEPO 周期治疗后，内皮祖细胞标志物显著增加，同时其凋亡率显著降低。rHuEPO 治疗前后的临床和实验室数据变化为 SSc 患者内皮干细胞成熟受损和新生血管形成缺陷的发病机制作用提供了新的见解。
