Patients with rare kidney diseases have higher rate of kidney failure
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Key takeaways:
- Patients with rare kidney diseases had a higher 5-year cumulative incidence of kidney failure than patients with all-cause CKD.
- Rare kidney diseases made up 5% to 10% of CKD cases.
Patients with rare kidney diseases have greater 5-year rates of kidney failure but higher survival than those with chronic kidney disease stages 3 through 5, according to published data.
Rare diseases generally affect “fewer than 200,000 individuals in the [United States] or fewer than five per 10,000 individuals in Europe,” Katie Wong, MBBS, of the department of renal medicine at the University College London, wrote with colleagues. “A better understanding of disease progression and of renal and patient survival is therefore needed, and could ... help to identify optimal opportunities for novel therapeutic interventions ... ”
In a longitudinal analysis using data from the U.K. National Registry of Rare Kidney Diseases, researchers evaluated 27,285 patients with 28 types of rare kidney diseases. The study included 108 U.K. renal care facilities between 2010 and 2022, with a median follow-up of 9.6 years. Main outcome was cumulative incidence of mortality and kidney failure.
Patients had higher 5-year cumulative incidence of kidney failure than 2.81 million U.K. patients with all-cause CKD (28% vs. 1%) but had better survival rates, the researchers found. Wong and colleagues also noted that median age at kidney failure and death, time from start of dialysis to death, time from diagnosis to eGFR thresholds, and the therapeutic trial window varied among different rare diseases.
Rare kidney diseases accounted for 5% to 10% of people with CKD, they wrote, but constituted more than 25% of patients receiving kidney replacement therapy.
“Although strategies to address cardiovascular risk and other causes of death are very important ... a substantial proportion of kidney failure is attributable to individually rare kidney diseases. Patients with rare kidney diseases should therefore be distinguished from those with more common causes of CKD, emphasizing the importance of early specialist referral and diagnosis.”
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