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Rare diseases

Progression and outcomes of rare kidney diseases

Nature Reviews Nephrology (2024)Cite this article

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To describe kidney and patient outcomes for individuals with rare diseases in the UK, Gale and colleagues analysed data from 27,285 patients with 28 different types of rare kidney disease in RaDaR. Over a median follow-up of 9.6 years, RaDaR participants had a significantly higher 5-year cumulative incidence of kidney failure than that of 2.81 million patients with all-cause CKD (28% versus 1%; P < 0.0001), but better survival (standardized mortality ratio 0.42; 95% CI 0.32–0.52; P < 0.0001). Despite substantial variation in measures such as age at kidney failure and death, the researchers found that the trajectory for patients with rare kidney diseases was faster than that of other patients with CKD. “This information might reframe perceptions of CKD, rare diseases and kidney failure among regulators, health-care providers and researchers,” concludes Gale.

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References

Original article

  • Wong, K. et al. Effects of rare kidney diseases on kidney failure: a longitudinal analysis of the UK National Registry of Rare Kidney Diseases (RaDaR) cohort. Lancet 403, 1279–1289 (2024)

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  1. Nature Reviews Nephrology http://www.nature.com/nrneph

    Susan J. Allison

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  1. Susan J. Allison
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Correspondence to Susan J. Allison.

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Allison, S.J. Progression and outcomes of rare kidney diseases. Nat Rev Nephrol (2024). https://doi.org/10.1038/s41581-024-00844-y

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