. 2024 Mar 30;403(10433):1279-1289.

doi: 10.1016/S0140-6736(23)02843-X. Epub 2024 Mar 13.

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

Katie Wong¹, David Pitcher², Fiona Braddon², Lewis Downward², Retha Steenkamp³, Nicholas Annear⁴, Jonathan Barratt⁵, Coralie Bingham⁶, Constantina Chrysochou⁷, Richard J Coward⁸, David Game⁹, Sian Griffin¹⁰, Matt Hall¹¹, Sally Johnson¹², Durga Kanigicherla⁷, Fiona Karet Frankl¹³, David Kavanagh¹⁴, Larissa Kerecuk¹⁵, Eamonn R Maher¹⁶, Shabbir Moochhala¹⁷, Jenny Pinney¹⁸, John A Sayer¹⁹, Roslyn Simms²⁰, Smeeta Sinha²¹, Shalabh Srivastava²², Frederick W K Tam²³, Andrew Neil Turner²⁴, Stephen B Walsh²⁵, Aoife Waters²⁶, Patricia Wilson²⁷, Edwin Wong²⁸, Christopher Mark Taylor¹⁵, Dorothea Nitsch²⁹, Moin Saleem⁸, Detlef Bockenhauer³⁰, Kate Bramham³¹, Daniel P Gale³²; RaDaR consortium

Collaborators, Affiliations

Collaborators

RaDaR consortium:
Sharirose Abat, Shazia Adalat, Joy Agbonmwandolor, Zubaidah Ahmad, Abdulfattah Alejmi, Rashid Almasarwah, Nicholas Annear, Ellie Asgari, Amanda Ayers, Jyoti Baharani, Gowrie Balasubramaniam, Felix Kpodo, Tarun Bansal, Alison Barratt, Jonathan Barratt, Megan Bates, Natalie Bayne, Janet Bendle, Sarah Benyon, Carsten Bergmann, Sunil Bhandari, Coralie Bingham, Preetham Boddana, Sally Bond, Fiona Braddon, Kate Bramham, Angela Branson, Stephen Brearey, Vicky Brocklebank, Sharanjit Budwal, Conor Byrne, Hugh Cairns, Brian Camilleri, Gary Campbell, Alys Capell, Margaret Carmody, Marion Carson, Tracy Cathcart, Christine Catley, Karine Cesar, Melanie Chan, Houda Chea, James Chess, Chee Kay Cheung, Katy-Jane Chick, Nihil Chitalia, Martin Christian, Tina Chrysochou, Katherine Clark, Christopher Clayton, Rhian Clissold, Helen Cockerill, Joshua Coelho, Elizabeth Colby, Viv Colclough, Eileen Conway, H Terence Cook, Wendy Cook, Theresa Cooper, Richard J Coward, Sarah Crosbie, Gabor Cserep, Anjali Date, Katherine Davidson, Amanda Davies, Neeraj Dhaun, Ajay Dhaygude, Lynn Diskin, Abhijit Dixit, Eunice Doctolero, Suzannah Dorey, Lewis Downard, Mark Drayson, Gavin Dreyer, Tina Dutt, Kufreabasi Etuk, Dawn Evans, Jenny Finch, Frances Flinter, James Fotheringham, Lucy Francis, Daniel P Gale, Hugh Gallagher, David Game, Eva Garcia, Madita Gavrila, Susie Gear, Colin Geddes, Mark Gilchrist, Matt Gittus, Paraskevi Goggolidou, Christopher Goldsmith, Patricia Gooden, Andrea Goodlife, Priyanka Goodwin, Tassos Grammatikopoulos, Barry Gray, Megan Griffith, Steph Gumus, Sanjana Gupta, Patrick Hamilton, Lorraine Harper, Tess Harris, Louise Haskell, Samantha Hayward, Shivaram Hegde, Bruce Hendry, Sue Hewins, Nicola Hewitson, Kate Hillman, Mrityunjay Hiremath, Alexandra Howson, Zay Htet, Sharon Huish, Richard Hull, Alister Humphries, David P J Hunt, Karl Hunter, Samantha Hunter, Marilyn Ijeomah-Orji, Nick Inston, David Jayne, Gbemisola Jenfa, Alison Jenkins, Sally Johnson, Caroline A Jones, Colin Jones, Amanda Jones, Rachel Jones, Lavanya Kamesh, Durga Kanigicherla, Fiona Karet Frankl, Mahzuz Karim, Amrit Kaur, David Kavanagh, Kelly Kearley, Larissa Kerecuk, Arif Khwaja, Garry King, Grant King, Ewa Kislowska, Edyta Klata, Maria Kokocinska, Mark Lambie, Laura Lawless, Thomas Ledson, Rachel Lennon, Adam P Levine, Ling Wai Maggie Lai, Graham Lipkin, Graham Lovitt, Paul Lyons, Holly Mabillard, Katherine Mackintosh, Khalid Mahdi, Eamonn Maher, Kevin J Marchbank, Patrick B Mark, Sherry Masoud, Bridgett Masunda, Zainab Mavani, Jake Mayfair, Stephen McAdoo, Joanna Mckinnell, Nabil Melhem, Simon Meyrick, Shabbir Moochhala, Putnam Morgan, Ann Morgan, Fawad Muhammad, Shona Murray, Kristina Novobritskaya, Albert Cm Ong, Louise Oni, Kate Osmaston, Neal Padmanabhan, Sharon Parkes, Jean Patrick, James Pattison, Riny Paul, Rachel Percival, Stephen J Perkins, Alexandre Persu, William G Petchey, Matthew C Pickering, Jennifer Pinney, David Pitcher, Lucy Plumb, Zoe Plummer, Joyce Popoola, Frank Post, Albert Power, Guy Pratt, Charles Pusey, Ria Rabara, May Rabuya, Tina Raju, Chadd Javier, Ian S D Roberts, Candice Roufosse, Adam Rumjon, Alan Salama, Moin Saleem, Richard Sandford, Kanwaljit S Sandu, Nadia Sarween, John A Sayer, Neil Sebire, Haresh Selvaskandan, Asheesh Sharma, Edward J Sharples, Neil Sheerin, Harish Shetty, Rukshana Shroff, Roslyn Simms, Manish Sinha, Smeeta Sinha, Kerry Smith, Lara Smith, Shalabh Srivastava, Retha Steenkamp, Ian Stott, Katerina Stroud, Pauline Swift, Justyna Szklarzewicz, Fred Tam, Kay Tan, Robert Taylor, Marc Tischkowitz, Kay Thomas, Yincent Tse, Alison Turnbull, A Neil Turner, Kay Tyerman, Miranda Usher, Gopalakrishnan Venkat-Raman, Alycon Walker, Stephen B Walsh, Aoife Waters, Angela Watt, Phil Webster, Ashutosh Wechalekar, Gavin I Welsh, Nicol West, David Wheeler, Kate Wiles, Lisa Willcocks, Angharad Williams, Emma Williams, Karen Williams, Deborah H Wilson, Patricia D Wilson, Paul Winyard, Edwin Wong, Katie Wong, Grahame Wood, Emma Woodward, Len Woodward, Adrian Woolf, David Wright

Affiliations

¹ National Registry of Rare Kidney Diseases, Bristol, UK; Department of Renal Medicine, University College London, London, UK.
² National Registry of Rare Kidney Diseases, Bristol, UK.
³ UK Renal Registry, Bristol, UK.
⁴ Institute of Medical and Biomedical Education, St George's University of London, London, UK.
⁵ Department of Cardiovascular Sciences, University of Leicester, Leicester, UK.
⁶ University of Exeter Medical School, University of Exeter, Exeter, UK.
⁷ Division of Cardiovascular Sciences, University of Manchester, Manchester, UK.
⁸ Translational Health Sciences, University of Bristol, Bristol, UK.
⁹ Guy's and St Thomas' NHS Foundation Trust, London, UK.
¹⁰ Department of Nephrology, University Hospital Wales, Cardiff, UK.
¹¹ Nottingham Renal and Transplant Unit, Nottingham University Hospitals NHS Foundation Trust, Nottingham, UK.
¹² Great North Children's Hospital, Newcastle upon Tyne, UK.
¹³ Cambridge Institute for Medical Research, University of Cambridge, Cambridge, UK.
¹⁴ National Renal Complement Therapeutics Centre, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK; Complement Therapeutics Research Group, Newcastle University, Newcastle upon Tyne, UK; Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK.
¹⁵ Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.
¹⁶ Department of Medical Genetics, University of Cambridge, Cambridge, UK.
¹⁷ Department of Renal Medicine, Royal Free London NHS Foundation Trust, London, UK.
¹⁸ Department of Renal Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
¹⁹ Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK.
²⁰ Academic Unit of Nephrology, Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.
²¹ Division of Cardiovascular Sciences, University of Manchester, Manchester, UK; Department of Renal Medicine, Northern Care Alliance NHS Foundation Trust, Manchester, UK.
²² Department of Renal Medicine, South Tyneside and Sunderland NHS Foundation Trust, Sunderland, UK.
²³ Centre for Inflammatory Disease, Department of Immunology and Inflammation, Imperial College London, London, UK.
²⁴ Medical Research Council Centre for Inflammation, Edinburgh University, Edinburgh, UK.
²⁵ Department of Renal Medicine, University College London, London, UK; Department of Renal Medicine, Royal Free London NHS Foundation Trust, London, UK.
²⁶ Department of Paediatrics and Child Health, University College Cork, Cork, Ireland.
²⁷ Department of Renal Medicine, University College London, London, UK.
²⁸ National Renal Complement Therapeutics Centre, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.
²⁹ UK Renal Registry, Bristol, UK; London School of Hygiene and Tropical Medicine, London, UK.
³⁰ Department of Renal Medicine, University College London, London, UK; Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
³¹ National Registry of Rare Kidney Diseases, Bristol, UK; King's Health Partners, King's College London, London, UK.
³² National Registry of Rare Kidney Diseases, Bristol, UK; Department of Renal Medicine, University College London, London, UK; Department of Renal Medicine, Royal Free London NHS Foundation Trust, London, UK. Electronic address: d.gale@ucl.ac.uk.

PMID: 38492578
PMCID: PMC11750427
DOI: 10.1016/S0140-6736(23)02843-X

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

Katie Wong et al. Lancet. 2024.

. 2024 Mar 30;403(10433):1279-1289.

doi: 10.1016/S0140-6736(23)02843-X. Epub 2024 Mar 13.

Authors

Collaborators

RaDaR consortium:
Sharirose Abat, Shazia Adalat, Joy Agbonmwandolor, Zubaidah Ahmad, Abdulfattah Alejmi, Rashid Almasarwah, Nicholas Annear, Ellie Asgari, Amanda Ayers, Jyoti Baharani, Gowrie Balasubramaniam, Felix Kpodo, Tarun Bansal, Alison Barratt, Jonathan Barratt, Megan Bates, Natalie Bayne, Janet Bendle, Sarah Benyon, Carsten Bergmann, Sunil Bhandari, Coralie Bingham, Preetham Boddana, Sally Bond, Fiona Braddon, Kate Bramham, Angela Branson, Stephen Brearey, Vicky Brocklebank, Sharanjit Budwal, Conor Byrne, Hugh Cairns, Brian Camilleri, Gary Campbell, Alys Capell, Margaret Carmody, Marion Carson, Tracy Cathcart, Christine Catley, Karine Cesar, Melanie Chan, Houda Chea, James Chess, Chee Kay Cheung, Katy-Jane Chick, Nihil Chitalia, Martin Christian, Tina Chrysochou, Katherine Clark, Christopher Clayton, Rhian Clissold, Helen Cockerill, Joshua Coelho, Elizabeth Colby, Viv Colclough, Eileen Conway, H Terence Cook, Wendy Cook, Theresa Cooper, Richard J Coward, Sarah Crosbie, Gabor Cserep, Anjali Date, Katherine Davidson, Amanda Davies, Neeraj Dhaun, Ajay Dhaygude, Lynn Diskin, Abhijit Dixit, Eunice Doctolero, Suzannah Dorey, Lewis Downard, Mark Drayson, Gavin Dreyer, Tina Dutt, Kufreabasi Etuk, Dawn Evans, Jenny Finch, Frances Flinter, James Fotheringham, Lucy Francis, Daniel P Gale, Hugh Gallagher, David Game, Eva Garcia, Madita Gavrila, Susie Gear, Colin Geddes, Mark Gilchrist, Matt Gittus, Paraskevi Goggolidou, Christopher Goldsmith, Patricia Gooden, Andrea Goodlife, Priyanka Goodwin, Tassos Grammatikopoulos, Barry Gray, Megan Griffith, Steph Gumus, Sanjana Gupta, Patrick Hamilton, Lorraine Harper, Tess Harris, Louise Haskell, Samantha Hayward, Shivaram Hegde, Bruce Hendry, Sue Hewins, Nicola Hewitson, Kate Hillman, Mrityunjay Hiremath, Alexandra Howson, Zay Htet, Sharon Huish, Richard Hull, Alister Humphries, David P J Hunt, Karl Hunter, Samantha Hunter, Marilyn Ijeomah-Orji, Nick Inston, David Jayne, Gbemisola Jenfa, Alison Jenkins, Sally Johnson, Caroline A Jones, Colin Jones, Amanda Jones, Rachel Jones, Lavanya Kamesh, Durga Kanigicherla, Fiona Karet Frankl, Mahzuz Karim, Amrit Kaur, David Kavanagh, Kelly Kearley, Larissa Kerecuk, Arif Khwaja, Garry King, Grant King, Ewa Kislowska, Edyta Klata, Maria Kokocinska, Mark Lambie, Laura Lawless, Thomas Ledson, Rachel Lennon, Adam P Levine, Ling Wai Maggie Lai, Graham Lipkin, Graham Lovitt, Paul Lyons, Holly Mabillard, Katherine Mackintosh, Khalid Mahdi, Eamonn Maher, Kevin J Marchbank, Patrick B Mark, Sherry Masoud, Bridgett Masunda, Zainab Mavani, Jake Mayfair, Stephen McAdoo, Joanna Mckinnell, Nabil Melhem, Simon Meyrick, Shabbir Moochhala, Putnam Morgan, Ann Morgan, Fawad Muhammad, Shona Murray, Kristina Novobritskaya, Albert Cm Ong, Louise Oni, Kate Osmaston, Neal Padmanabhan, Sharon Parkes, Jean Patrick, James Pattison, Riny Paul, Rachel Percival, Stephen J Perkins, Alexandre Persu, William G Petchey, Matthew C Pickering, Jennifer Pinney, David Pitcher, Lucy Plumb, Zoe Plummer, Joyce Popoola, Frank Post, Albert Power, Guy Pratt, Charles Pusey, Ria Rabara, May Rabuya, Tina Raju, Chadd Javier, Ian S D Roberts, Candice Roufosse, Adam Rumjon, Alan Salama, Moin Saleem, Richard Sandford, Kanwaljit S Sandu, Nadia Sarween, John A Sayer, Neil Sebire, Haresh Selvaskandan, Asheesh Sharma, Edward J Sharples, Neil Sheerin, Harish Shetty, Rukshana Shroff, Roslyn Simms, Manish Sinha, Smeeta Sinha, Kerry Smith, Lara Smith, Shalabh Srivastava, Retha Steenkamp, Ian Stott, Katerina Stroud, Pauline Swift, Justyna Szklarzewicz, Fred Tam, Kay Tan, Robert Taylor, Marc Tischkowitz, Kay Thomas, Yincent Tse, Alison Turnbull, A Neil Turner, Kay Tyerman, Miranda Usher, Gopalakrishnan Venkat-Raman, Alycon Walker, Stephen B Walsh, Aoife Waters, Angela Watt, Phil Webster, Ashutosh Wechalekar, Gavin I Welsh, Nicol West, David Wheeler, Kate Wiles, Lisa Willcocks, Angharad Williams, Emma Williams, Karen Williams, Deborah H Wilson, Patricia D Wilson, Paul Winyard, Edwin Wong, Katie Wong, Grahame Wood, Emma Woodward, Len Woodward, Adrian Woolf, David Wright

Affiliations

¹ National Registry of Rare Kidney Diseases, Bristol, UK; Department of Renal Medicine, University College London, London, UK.
² National Registry of Rare Kidney Diseases, Bristol, UK.
³ UK Renal Registry, Bristol, UK.
⁴ Institute of Medical and Biomedical Education, St George's University of London, London, UK.
⁵ Department of Cardiovascular Sciences, University of Leicester, Leicester, UK.
⁶ University of Exeter Medical School, University of Exeter, Exeter, UK.
⁷ Division of Cardiovascular Sciences, University of Manchester, Manchester, UK.
⁸ Translational Health Sciences, University of Bristol, Bristol, UK.
⁹ Guy's and St Thomas' NHS Foundation Trust, London, UK.
¹⁰ Department of Nephrology, University Hospital Wales, Cardiff, UK.
¹¹ Nottingham Renal and Transplant Unit, Nottingham University Hospitals NHS Foundation Trust, Nottingham, UK.
¹² Great North Children's Hospital, Newcastle upon Tyne, UK.
¹³ Cambridge Institute for Medical Research, University of Cambridge, Cambridge, UK.
¹⁴ National Renal Complement Therapeutics Centre, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK; Complement Therapeutics Research Group, Newcastle University, Newcastle upon Tyne, UK; Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK.
¹⁵ Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.
¹⁶ Department of Medical Genetics, University of Cambridge, Cambridge, UK.
¹⁷ Department of Renal Medicine, Royal Free London NHS Foundation Trust, London, UK.
¹⁸ Department of Renal Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
¹⁹ Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK.
²⁰ Academic Unit of Nephrology, Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.
²¹ Division of Cardiovascular Sciences, University of Manchester, Manchester, UK; Department of Renal Medicine, Northern Care Alliance NHS Foundation Trust, Manchester, UK.
²² Department of Renal Medicine, South Tyneside and Sunderland NHS Foundation Trust, Sunderland, UK.
²³ Centre for Inflammatory Disease, Department of Immunology and Inflammation, Imperial College London, London, UK.
²⁴ Medical Research Council Centre for Inflammation, Edinburgh University, Edinburgh, UK.
²⁵ Department of Renal Medicine, University College London, London, UK; Department of Renal Medicine, Royal Free London NHS Foundation Trust, London, UK.
²⁶ Department of Paediatrics and Child Health, University College Cork, Cork, Ireland.
²⁷ Department of Renal Medicine, University College London, London, UK.
²⁸ National Renal Complement Therapeutics Centre, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.
²⁹ UK Renal Registry, Bristol, UK; London School of Hygiene and Tropical Medicine, London, UK.
³⁰ Department of Renal Medicine, University College London, London, UK; Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
³¹ National Registry of Rare Kidney Diseases, Bristol, UK; King's Health Partners, King's College London, London, UK.
³² National Registry of Rare Kidney Diseases, Bristol, UK; Department of Renal Medicine, University College London, London, UK; Department of Renal Medicine, Royal Free London NHS Foundation Trust, London, UK. Electronic address: d.gale@ucl.ac.uk.

PMID: 38492578
PMCID: PMC11750427
DOI: 10.1016/S0140-6736(23)02843-X

Erratum in

Department of Error.
[No authors listed] [No authors listed] Lancet. 2025 Apr 5;405(10485):1146. doi: 10.1016/S0140-6736(25)00622-1. Lancet. 2025. PMID: 40187841 No abstract available.

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.

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Conflict of interest statement

Declaration of interests ERM reports support for the current manuscript from VHL UK/Ireland and consulting fees from MSD. SM is chair of OxalEurope. MS reports support for the current manuscript from a Medical Research Council UK Precision Medicine programme grant (MR/R013942/1) and consulting fees from Travere Therapeutics. RJC reports support for the current manuscript from Kidney Research UK. JAS reports support for the current manuscript from Kidney Research UK, Northern Counties Kidney Research Fund, and the Medical Research Council UK (all payments to institution). JAS is Academic Vice President of the UK Kidney Association. FWKT reports support from the National Institute for Health and Care Research Imperial Biomedical Centre. DN is the UK Kidney Association Director of Informatics Research. DPG reports support for the current manuscript from St Peter's Trust for Kidney Bladder and Prostate Research, Medical Research Council, Kidney Research UK, Kidney Care UK, and Polycystic Kidney Disease Charity (all payments to institution). DPG chairs the Rare Diseases Committee of the UK Kidney Association and reports fees for consulting and presenting from Novartis, Alexion, Calliditas, Sanofi, Britannia, and Travere. All other authors declare no competing interests.

Figures

**Figure 1**
Kaplan-Meier estimates of cumulative incidence of kidney failure for glomerular (A) and cystic kidney diseases (B) Data for all diseases are shown in the appendix (p 4). Data are censored for death. ADPKD=autosomal dominant polycystic kidney disease. ADTKD=autosomal dominant tubulointerstitial kidney disease. ANCA=antineutrophil cytoplasmic antibody. eGFR=estimated glomerular filtration rate. MPGN–C3G=membranoproliferative glomerulonephritis and C3 glomerulopathy. SRNS–FSGS=steroid resistant nephrotic syndrome, congenital nephrotic syndrome, or focal segmental glomerulosclerosis. SSNS–MCD=steroid sensitive nephrotic syndrome or minimal change disease.

**Figure 2**
Kaplan-Meier estimates of median time from diagnosis to eGFR value for glomerular (A) and cystic kidney diseases (B) Data for all diseases are shown in the appendix (p 8). ADPKD=autosomal dominant polycystic kidney disease. ADTKD=autosomal dominant tubulointerstitial kidney disease. ANCA=antineutrophil cytoplasmic antibody. eGFR=estimated glomerular filtration rate. GBM=glomerular basement membrane. MPGN–C3G=membranoproliferative glomerulonephritis and C3 glomerulopathy. SRNS–FSGS=steroid resistant nephrotic syndrome, congenital nephrotic syndrome, or focal segmental glomerulosclerosis. SSNS–MCD=steroid sensitive nephrotic syndrome or minimal change disease.

**Figure 3**
Observed vs predicted kidney failure within 2 years in RaDaR patients Patients with eGFR >15 and <60 mL/min per 1·73 m² at recruitment who remained alive with more than 2 years of follow-up data are shown. Observed kidney failure exceeded that predicted by the KFRE (Hosmer-Lemeshow test; p<0·0001). Data stratified by age group are shown in the appendix (p 12). Blue dashed line indicates observed proportion of kidney failure within 2 years equalling the predicted risk score. KFRE=kidney failure risk equation. RaDaR=the National Registry of Rare Kidney Diseases.

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Comment in

The impact of rare kidney diseases on kidney failure.
Aiyegbusi OL, Fenton A. Aiyegbusi OL, et al. Lancet. 2024 Mar 30;403(10433):1211-1213. doi: 10.1016/S0140-6736(24)00198-3. Epub 2024 Mar 13. Lancet. 2024. PMID: 38492577 No abstract available.
Progression and outcomes of rare kidney diseases.
Allison SJ. Allison SJ. Nat Rev Nephrol. 2024 Jun;20(6):353. doi: 10.1038/s41581-024-00844-y. Nat Rev Nephrol. 2024. PMID: 38671191 No abstract available.

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

Collaborators

Affiliations

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

Authors

Collaborators

Affiliations

Erratum in

Abstract

Conflict of interest statement

Figures

Comment in

References

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