Aurélie Lavergne1,2, Adelaïde Pladys3, Cécile Couchoud4, Mathilde Lassalle4, Cécile Vigneau5,6,7. 1. Université Rennes1, 35043, Rennes, France. aurelie.lavergne@chu-rennes.fr. 2. CHU Rennes, Service de Néphrologie, 35033, Rennes, France. aurelie.lavergne@chu-rennes.fr. 3. EHESP Rennes, Sorbonne Paris Cité, EA 7449, REPERES, Rennes, France. 4. REIN Registry, Agence de la Biomédecine, Saint Denis La Plaine, France. 5. Université Rennes1, 35043, Rennes, France. 6. CHU Rennes, Service de Néphrologie, 35033, Rennes, France. 7. INSERM, U1085 IRSET-9, 35033, Rennes, France.
Abstract
BACKGROUND: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
BACKGROUND: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). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.