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Systemic sclerosis and end-stage renal disease: study of patient characteristics, follow-up and outcomes in France

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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.

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Acknowledgements

Authors thank REIN Registry and all its investigators (www.agence-biomedecine.fr) for the data collection and access. We thank Angela Swain-Verdier for revising the English. The authors declare that the results presented in this paper have not been published previously in whole or in part, except in abstract format.

Funding

This research received funding from the Agence de la Biomédecine.

Author information

Authors and Affiliations

  1. Université Rennes1, 35043, Rennes, France

    Aurélie Lavergne & Cécile Vigneau

  2. CHU Rennes, Service de Néphrologie, 35033, Rennes, France

    Aurélie Lavergne & Cécile Vigneau

  3. EHESP Rennes, Sorbonne Paris Cité, EA 7449, REPERES, Rennes, France

    Adelaïde Pladys

  4. REIN Registry, Agence de la Biomédecine, Saint Denis La Plaine, France

    Cécile Couchoud & Mathilde Lassalle

  5. INSERM, U1085 IRSET-9, 35033, Rennes, France

    Cécile Vigneau

Authors
  1. Aurélie Lavergne
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  2. Adelaïde Pladys
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  3. Cécile Couchoud
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  4. Mathilde Lassalle
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  5. Cécile Vigneau
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Contributions

AL collected the data from medical forms, designed the study, and wrote the paper. AP performed the statistical analysis and contributed to the writing. CC contributed to design the study, and helped to analysis tools and data from REIN registry. ML performed the extraction of included patients and data from REIN database, performed the matching of patients with controls. CV conceived the original idea, contributed to design the study and supervised the work. All authors proofread the paper and approved the final version of manuscript.

Corresponding author

Correspondence to Aurélie Lavergne.

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Nothing to declare.

Ethical approval

The National Commission for Information Technology and Privacy (CNIL) approved the data collection conducted by the REIN. The REIN is registered with the CNIL under the number 903188 Version 3. The REIN Scientific Committee approved this study.

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Lavergne, A., Pladys, A., Couchoud, C. et al. Systemic sclerosis and end-stage renal disease: study of patient characteristics, follow-up and outcomes in France. J Nephrol 34, 617–625 (2021). https://doi.org/10.1007/s40620-020-00746-9

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  • DOI: https://doi.org/10.1007/s40620-020-00746-9

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