Lavergne Aurélie, Pladys Adelaïde, Couchoud Cécile, Lassalle Mathilde, Vigneau Cécile
Université Rennes1, 35043, Rennes, France.
CHU Rennes, Service de Néphrologie, 35033, Rennes, France.
J Nephrol. 2021 Apr;34(2):617-625. doi: 10.1007/s40620-020-00746-9. Epub 2020 May 24.
Scleroderma renal crisis (SRC), the most frequent renal complication of Systemic Sclerosis (SSc), can lead to end-stage renal disease (ESRD), most frequently, but not exclusively, because of scleroderma renal crisis (SRC).
The main objectives of our study using data extracted from the French renal epidemiology and information network (REIN) registry, were to describe the characteristics and outcomes in an incident French cohort of SSc patients requiring renal replacement therapy (RRT) compared with a matched RRT patient sample.
Between 2002 and 2014, 120 incident SSc patients started RRT in France. SSc was significantly associated with higher mortality (HR 1.95; 95% CI 1.41-2.71; p = 0.001) in comparison with matched controls. Among SSc patients in dialysis, besides age, the only risk factor independently associated with mortality was the inability to walk without help (HR 2.34, CI 95% 1.37-4.02, p = 0.002). Dialysis withdrawal was reported for 22 (18.3%) of the SSc patients compared to 15 (6.3%) for the controls. Patients with SSc have less access to transplantation waiting list (HR 0.21; CI 95% 0.11-0.41, p < 0.001) and to kidney transplantation (KTR) (HR 0.22; 95% CI 0.12-0.43; p < 0.001). During the follow-up, 6 of the 27 patients (22.2%) registered on KTR waiting list died compared to 69 of the 93 (74.2%) patients who were not on the waiting list.
The prognosis for SSc patients requiring RRT is still poor, with a significantly higher mortality and lower registration on kidney transplant waiting-list compared to matched controls.
硬皮病肾危象（SRC）是系统性硬化症（SSc）最常见的肾脏并发症，可导致终末期肾病（ESRD），最常见但并非唯一的原因是硬皮病肾危象（SRC）。
我们利用从法国肾脏流行病学和信息网络（REIN）登记处提取的数据进行研究，主要目的是描述法国一组需要肾脏替代治疗（RRT）的SSc患者与匹配的RRT患者样本相比的特征和结局。
2002年至2014年期间，法国有120例新发病的SSc患者开始接受RRT。与匹配的对照组相比，SSc与更高的死亡率显著相关（HR 1.95；95%CI 1.41 - 2.71；p = 0.001）。在接受透析的SSc患者中，除年龄外，唯一与死亡率独立相关的危险因素是无法独立行走（HR 2.34，95%CI 1.37 - 4.02，p = 0.002）。报告有22例（18.3%）SSc患者停止透析，而对照组为15例（6.3%）。SSc患者进入移植等待名单（HR 0.21；95%CI 0.11 - 0.41，p < 0.001）和接受肾脏移植（KTR）（HR 0.22；95%CI 0.12 - 0.43；p < 0.001）的机会较少。在随访期间，登记在KTR等待名单上的27例患者中有6例（22.2%）死亡，而未在等待名单上的93例患者中有69例（74.2%）死亡。
需要RRT的SSc患者预后仍然很差，与匹配的对照组相比，死亡率显著更高，进入肾脏移植等待名单的比例更低。
