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Abstract: SA-OR81

Unveiling the Disproportionate Impact of Rare Kidney Diseases on Kidney Failure: A Longitudinal Analysis Using the UK National Registry of Rare Kidney Diseases (RaDaR)

Session Information

Category: Genetic Diseases of the Kidneys

  • 1202 Genetic Diseases of the Kidneys: Non-Cystic


  • Gale, Daniel P., Rare Renal Disease Registry, Bristol, United Kingdom
  • Wong, Katie, Rare Renal Disease Registry, Bristol, United Kingdom
  • Pitcher, David, Rare Renal Disease Registry, Bristol, United Kingdom
  • Nitsch, Dorothea, London School of Hygiene & Tropical Medicine Faculty of Epidemiology and Population Health, London, United Kingdom
  • Bramham, Kate, Rare Renal Disease Registry, Bristol, United Kingdom

Patients with rare kidney diseases account for <10% of people living with CKD, but make up 25% of prevalent patients on Renal Replacement Therapy (RRT). The natural history of rare kidney diseases are poorly described. Formed in 2010, RaDaR gathers longitudinal data from UK rare kidney disease patients. We used these data to study outcomes of death and RRT initiation for these conditions.


RaDaR recruited patients from 108 UK renal units. Incidence rates for mortality and ESKD were calculated and compared to those from unselected UK patients using population estimates of CKD and UK Renal Registry RRT incidence data. Cumulative incidence and Kaplan Meier survival estimates were calculated for a)Age at RRT start b)age at death c)time from RRT start to death d)time from diagnosis to eGFR thresholds allowing calculation of time from last eGFR ≥75 to first <30mls/min/1.73m2 (therapeutic window).


27,293 patients in 20 Rare Disease Groups (RDGs) were included. RaDaR patients had higher 5 year incidence rates of ESKD compared to 2.81 million patients with all cause CKD (28% vs 1%) and better survival rates (Standardised Mortality Ratio 0.45, 95% CI 0.37-0.54). There was heterogeneity in median age at RRT start between RDGs (Fig 1). Time in therapeutic window varied between 20 years in Retroperitoneal Fibrosis to 1.3 years in Monoclonal Gammopathy of Renal Significance.


Patients with rare kidney diseases differ from the general CKD population: they are more likely to reach ESKD and half as likely to die with CKD stages 3-5, so are disproportionately represented in the RRT population. Successfully addressing unmet need in rare diseases may therefore have a disproportionate effect on RRT demand long term.