Mandelbrot Didier A, Santos Peter W, Burt Richard K, Oyama Yu, Block Geoffrey A, Ahya Shubhada N, Rosa Robert M, Traynor Ann E
Division of Nephrology, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA.
Nephrol Dial Transplant. 2008 Aug;23(8):2679-84. doi: 10.1093/ndt/gfn036. Epub 2008 Mar 7.
Calciphylaxis and calcinosis can both cause severe morbidity and mortality in patients with systemic lupus erythematosus (SLE). Haematopoietic stem cell transplantation (HSCT) has been successfully used to treat patients with refractory SLE. It was hypothesized that in calciphylaxis and calcinosis, ongoing inflammatory activity contributes to the calcium deposition in the media of small arteries, as well as perivascular and periarticular tissues. We report three patients whose soft-tissue calcification syndromes dramatically resolved after undergoing HSCT.
Three patients referred for refractory SLE underwent HSCT at a tertiary care medical center. SLE serologies and clinical features before and after HSCT were recorded.
Despite receiving >6 months of intravenous cyclophosphamide (CYC), three SLE patients showed signs of persistent lupus activity, including severe soft-tissue calcification. The first patient was on haemodialysis and developed severe calciphylaxis with large ulcers and tissue necrosis. The second patient had calcinosis, with palpable crystals extruding from ulcers. The third patient had calcinosis characterized by subcutaneous nodules and plaques. Because prior conventional therapies had failed, the three were treated with high-dose CYC, anti-thymocyte globulin and HSCT. They have been followed post-HSCT for 26-38 months, with excellent clinical responses, including sustained resolution of skin abnormalities.
The successful treatment of advanced calcium deposition by aggressive immune ablation underscores the contribution of SLE-mediated inflammation to soft-tissue calcification syndromes.
钙化防御和钙质沉着症均可导致系统性红斑狼疮（SLE）患者出现严重的发病率和死亡率。造血干细胞移植（HSCT）已成功用于治疗难治性SLE患者。据推测，在钙化防御和钙质沉着症中，持续的炎症活动会导致小动脉中层以及血管周围和关节周围组织中的钙沉积。我们报告了三名患者，他们在接受HSCT后软组织钙化综合征得到了显著缓解。
三名因难治性SLE前来就诊的患者在一家三级医疗中心接受了HSCT。记录了HSCT前后的SLE血清学和临床特征。
尽管接受了超过6个月的静脉注射环磷酰胺（CYC），但三名SLE患者仍表现出持续的狼疮活动迹象，包括严重的软组织钙化。第一名患者正在接受血液透析，并出现了严重的钙化防御，伴有大面积溃疡和组织坏死。第二名患者患有钙质沉着症，有从溃疡中挤出的可触及晶体。第三名患者患有以皮下结节和斑块为特征的钙质沉着症。由于先前的传统疗法均告失败，这三名患者接受了大剂量CYC、抗胸腺细胞球蛋白和HSCT治疗。HSCT后对他们进行了26至38个月的随访，临床反应良好，包括皮肤异常持续消退。
通过积极的免疫消融成功治疗晚期钙沉积强调了SLE介导的炎症对软组织钙化综合征的作用。
