Effects of rare kidney diseases on kidney failure: a longitudinal analysis of the UK National Registry of Rare Kidney Diseases (RaDaR) cohort

Abstract

Background: Individuals with rare kidney diseases account for 5–10% of people with chronic kidney disease, but constitute more than 25% of patients receiving kidney replacement therapy. The National Registry of Rare Kidney Diseases (RaDaR) gathers longitudinal data from patients with these conditions, which we used to study disease progression and outcomes of death and kidney failure. Methods: People aged 0–96 years living with 28 types of rare kidney diseases were recruited from 108 UK renal care facilities. The primary outcomes were cumulative incidence of mortality and kidney failure in individuals with rare kidney diseases, which were calculated and compared with that of unselected patients with chronic kidney disease. Cumulative incidence and Kaplan–Meier survival estimates were calculated for the following outcomes: median age at kidney failure; median age at death; time from start of dialysis to death; and time from diagnosis to estimated glomerular filtration rate (eGFR) thresholds, allowing calculation of time from last eGFR of 75 mL/min per 1·73 m2 or more to first eGFR of less than 30 mL/min per 1·73 m2 (the therapeutic trial window). Findings: Between Jan 18, 2010, and July 25, 2022, 27 285 participants were recruited to RaDaR. Median follow-up time from diagnosis was 9·6 years (IQR 5·9–16·7). RaDaR participants had significantly higher 5-year cumulative incidence of kidney failure than 2·81 million UK patients with all-cause chronic kidney disease (28% vs 1%; p<0·0001), but better survival rates (standardised mortality ratio 0·42 [95% CI 0·32–0·52]; p<0·0001). Median age at kidney failure, median age at death, time from start of dialysis to death, time from diagnosis to eGFR thresholds, and therapeutic trial window all varied substantially between rare diseases. Interpretation: Patients with rare kidney diseases differ from the general population of individuals with chronic kidney disease: they have higher 5-year rates of kidney failure but higher survival than other patients with chronic kidney disease stages 3–5, and so are over-represented in the cohort of patients requiring kidney replacement therapy. Addressing unmet therapeutic need for patients with rare kidney diseases could have a large beneficial effect on long-term kidney replacement therapy demand. Funding: RaDaR is funded by the Medical Research Council, Kidney Research UK, Kidney Care UK, and the Polycystic Kidney Disease Charity.

Publication DOI: https://doi.org/10.1016/S0140-6736(23)02843-X
Divisions: College of Health & Life Sciences > Aston Medical School
Funding Information: The authors thank all RaDaR participants, their family members, and the UK Renal Registry staff. RaDaR is funded by the UK Medical Research Council, Kidney Research UK, Kidney Care UK, and the Polycystic Kidney Disease Charity.
Additional Information: Copyright © 2024 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY 4.0 license.
Uncontrolled Keywords: Humans,Glomerular Filtration Rate,Kidney,Kidney Failure, Chronic/epidemiology,Radar,Rare Diseases,Registries,Renal Insufficiency/epidemiology,Renal Insufficiency, Chronic/epidemiology,United Kingdom/epidemiology,Infant, Newborn,Infant,Child, Preschool,Child,Adolescent,Young Adult,Adult,Middle Aged,Aged,Aged, 80 and over,General Medicine
Publication ISSN: 1474-547X
Data Access Statement: The RaDaR database is hosted by the UK Renal Registry and its metadata are available via https://rarerenal.org. Individual-level data are not available for export. Proposals to perform analyses using the data for academic, audit, or commercial purposes can be made to the RaDaR Operations Group via https://rarerenal.org.
Last Modified: 08 Nov 2024 08:26
Date Deposited: 25 Jul 2024 16:26
Full Text Link:
Related URLs: https://www.sci ... 14067362302843X (Publisher URL)
http://www.scop ... tnerID=8YFLogxK (Scopus URL)
PURE Output Type: Article
Published Date: 2024-03-30
Published Online Date: 2024-03-13
Accepted Date: 2024-03-01
Authors: Wong, Katie
Pitcher, David
Braddon, Fiona
Downward, Lewis
Steenkamp, Retha
Annear, Nicholas
Barratt, Jonathan
Bingham, Coralie
Chrysochou, Constantina
Coward, Richard J.
Game, David
Griffin, Sian
Hall, Matt
Johnson, Sally
Kanigicherla, Durga
Karet Frankl, Fiona
Kavanagh, David
Kerecuk, Larissa
Maher, Eamonn
Moochhala, Shabbir
Pinney, Jenny
Sayer, John A.
Simms, Roslyn
Sinha, Smeeta
Srivastava, Shalabh
Tam, Frederick W.K.
Turner, Andrew Neil
Walsh, Stephen B.
Waters, Aoife
Wilson, Patricia
Wong, Edwin
Taylor, Christopher Mark
Nitsch, Dorothea
Saleem, Moin
Bockenhauer, Detlef
Bramham, Kate
Gale, Daniel P.

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