Gibney Eric M, Parikh Chirag R, Jani Alkesh, Fischer Michael J, Collier David, Wiseman Alexander C
Division of Renal Diseases and Hypertension, University of Colorado Health Sciences Center, Denver, CO, USA.
Am J Transplant. 2004 Dec;4(12):2027-31. doi: 10.1111/j.1600-6143.2004.00605.x.
Systemic sclerosis (SS) may lead to sclerodema renal crisis, an unusual cause of end-stage renal disease (ESRD) with historically poor hemodialysis outcomes. Little information is available on outcomes after kidney transplantation. Information from the UNOS registry was obtained on SS patients in the United States, listed for kidney transplants between 1985-2002. We compared survival at 1 and 3 years in patients who received cadaveric transplants with patients who remained on the waiting list. Graft survival, cause of graft loss, frequency of early graft loss and pre- and post-transplant skin scores were analyzed. Two hundred and fifty-eight patients with SS were listed for transplantation. Survival was significantly prolonged in patients receiving transplants (p = 0.005). Graft survival at 1 and 3 years was 68% and 60%. Early graft loss was common. Skin scores improved in all four subjects at our center, with an average decline of 60.7% (p = 0.024). Kidney transplantation confers a survival benefit in ESRD due to SS. Transplantation may be associated with an improvement in systemic manifestations of disease. Despite suboptimal graft survival, kidney transplant should be considered the treatment of choice in ESRD due to SS.
系统性硬化症（SS）可能导致硬皮病肾危象，这是终末期肾病（ESRD）的一种罕见病因，历来血液透析效果不佳。关于肾移植后的预后信息较少。我们从美国器官共享联合网络（UNOS）登记处获取了1985年至2002年间登记等待肾移植的SS患者的信息。我们比较了接受尸体肾移植患者与仍在等待名单上患者1年和3年的生存率。分析了移植物存活率、移植物丢失原因、早期移植物丢失频率以及移植前后的皮肤评分。共有258例SS患者登记等待移植。接受移植的患者生存率显著延长（p = 0.005）。1年和3年的移植物存活率分别为68%和60%。早期移植物丢失很常见。我们中心的所有4名受试者皮肤评分均有改善，平均下降60.7%（p = 0.024）。肾移植可使因SS导致的ESRD患者获得生存益处。移植可能与疾病的全身表现改善有关。尽管移植物存活率不理想，但肾移植仍应被视为因SS导致的ESRD的首选治疗方法。
