Nephrology, Dialysis and Renal Transplant Unit, IRCCS-Azienda Ospedaliero-Universitaria di Bologna, Alma Mater Studiorum University of Bologna, Bologna, Italy.
Front Immunol. 2022 Jul 26;13:878736. doi: 10.3389/fimmu.2022.878736. eCollection 2022.
Systemic sclerosis (SSc) is an immune-mediated rheumatic disease characterized by vascular abnormalities, tissue fibrosis, and inflammation. Renal disease occurring in patients with SSc may have a variable clinicopathological picture. However, the most specific renal condition associated with this disease is the scleroderma renal crisis (SRC), characterized by acute onset of renal failure and severe hypertension. SRC develops in about 20% of cases of SSc, especially in those patients with diffuse cutaneous disease. The prognosis of this condition is often negative, with a rapid progression to end-stage renal disease (ESRD). The advent of the antihypertensive angiotensin-converting enzyme inhibitors in 1980 was associated with a significant improvement in patients' survival and recovery of renal function. However, the prognosis of these patients can still be improved. The dialytic condition is associated with early death, and mortality is significantly higher than among patients undergoing renal replacement therapy (RRT) due to other conditions. Patients with SRC who show no signs of renal functional recovery despite timely blood pressure control are candidates for kidney transplantation (KT). In this review, we reported the most recent advances in KT in patients with ESRD due to SSc, with a particular overview of the risk of disease recurrence after transplantation and the evolution of other disease manifestations.
系统性硬化症（SSc）是一种免疫介导的风湿性疾病，其特征为血管异常、组织纤维化和炎症。发生于 SSc 患者的肾脏疾病可能具有不同的临床病理表现。然而，与该疾病最相关的特定肾脏情况是硬皮病肾危象（SRC），其特征为肾功能衰竭和严重高血压的急性发作。SRC 在大约 20%的 SSc 病例中发生，尤其是在那些弥漫性皮肤疾病患者中。这种情况的预后通常较差，迅速进展为终末期肾病（ESRD）。1980 年，血管紧张素转换酶抑制剂的降压作用问世，这与患者的生存和肾功能恢复显著改善相关。然而，这些患者的预后仍可得到改善。透析情况与早期死亡相关，死亡率明显高于因其他情况接受肾脏替代治疗（RRT）的患者。尽管及时控制血压，但仍未显示肾功能恢复迹象的 SRC 患者是肾移植（KT）的候选者。在这篇综述中，我们报告了 ESRD 患者 KT 的最新进展，特别概述了移植后疾病复发的风险和其他疾病表现的演变。
