Progression and outcomes of rare kidney diseases

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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