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Lookup NU author(s): Laura Bailey, Vikki Bridgett, Dr Vicky Brocklebank, Dr Sally Johnson, Professor David KavanaghORCiD, Dr Holly MabillardORCiD, Professor Kevin MarchbankORCiD, Professor John SayerORCiD, Professor Neil SheerinORCiD, Professor Robert TaylorORCiD, Dr Yincent TseORCiD, Dr Edwin Wong
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© 2025 International Society of Nephrology. Introduction: C3 glomerulopathy (C3G) and immune-complex membranoproliferative glomerulonephritis (IC-MPGN) are rare disorders that frequently result in kidney failure over the long-term. Presently, there are no disease-specific treatments approved for these disorders, although there is much interest in the therapeutic potential of complement inhibition. However, the limited duration and necessarily small size of controlled trials means there is a need to quantify how well short-term changes in estimated glomerular filtration rate (eGFR) and proteinuria predict the clinically important outcome of kidney failure. Methods: We address this using longitudinal data from the UK Registry of Rare Kidney Diseases (RaDaR) involving retrospective and prospective data collection with linkage to hospital laboratories via automated feeds of 371 patients. Analyses of kidney survival were conducted using Kaplan–Meier and Cox regression with eGFR slope estimated using linear mixed models. Results: In a median of 11.0 (inter quartile range 7.4-15.1) years follow-up, 148 patients (40%) reached kidney failure. There was no significant difference in progression to kidney failure between C3G and IC-MPGN groups. Baseline urine protein-creatinine ratio (UPCR), although high, was not associated with kidney failure in either group. Two-year eGFR slope had a modest association with kidney failure. In contrast, both 20%‒50% and 50 mg/mmol reductions in UPCR between 0-12 months were associated with lower kidney failure risk in both groups. Notably, those with a UPCR under 100 mg/mmol at 12 months had a substantially lower risk of kidney failure (hazard ratio 0.10 (95% confidence interval 0.03-0.30). Conclusions: Overall, proteinuria a short time after diagnosis is strongly associated with long-term outcomes and a UPCR under 100 mg/mmol at one year is associated with a substantially lower kidney failure risk.
Author(s): Masoud S, Wong K, Pitcher D, Downward L, Proudfoot C, Webb NJA, Abat S, Adalat S, Agbonmwandolor J, Ahmad Z, Alejmi A, Almasarwah R, Annear N, Araujo M, Asgari E, Ayers A, Baharani J, Balasubramaniam G, Jo-Bamba Kpodo F, Bailey L, Bansal T, Barratt A, Barratt J, Bates M, Bayne N, Bendle J, Benyon S, Bergmann C, Bhandari S, Bingham C, Boddana P, Bond S, Braddon F, Bramham K, Branson A, Brearey S, Bridgett V, Brocklebank V, Budwal S, Byrne C, Cairns H, Camilleri B, Campbell G, Capell A, Carmody M, Carson M, Cathcart T, Catley C, Cesar K, Chan M, Chea H, Chess J, Cheung CK, Chick K-J, Chitalia N, Christian M, Chrysochou T, Clark K, Clayton C, Clissold R, Cockerill H, Coelho J, Colby E, Colclough V, Conway E, Cook HT, Cook W, Cooper T, Coward RJ, Crosbie S, Cserep G, Date A, Davidson K, Davies A, Dhaun N, Dhaygude A, Diskin L, Dixit A, Doctolero EA, Dorey S, Downard L, Drayson M, Dreyer G, Dutt T, Etuk K, Evans D, Finch J, Flinter F, Fotheringham J, Francis L, Gale DP, Gale DP, Gallagher H, Game D, Garcia EL, Gavrila M, Gear S, Geddes C, Gilchrist M, Gittus M, Goggolidou P, Goldsmith C, Gooden P, Goodlife A, Goodwin P, Grammatikopoulos T, Gray B, Griffith M, Gumus S, Gupta S, Guzman R, Hamilton P, Hamrang-Yousefi S, Harper L, Harris T, Haskell L, Hayward S, Hegde S, Hendry B, Hewins S, Hewitson N, Hillman K, Hiremath M, Howson A, Htet Z, Huish S, Hull R, Humphries A, Hunt DPJ, Hunter K, Hunter S, Ijeomah-Orji M, Inston N, Jayne D, Jenfa G, Jenkins A, Johnson S, Jones CA, Jones C, Jones A, Jones R, Kamesh L, Kanigicherla D, Frankl FK, Karim M, Kaur A, Kavanagh D, Kearley K, Kerecuk L, Khwaja A, King G, King G, Kislowska E, Klata E, Kokocinska M, Koziell A, Lambie M, Lawless L, Ledson T, Lennon R, Levine AP, Maggie Lai LW, Lipkin G, Lovitt G, Lyons P, Mabillard H, Mackintosh K, Mahdi K, Maher E, Marchbank KJ, Mark PB, Masoud S, Masunda B, Mavani Z, Mayfair J, McAdoo S, Mckinnell J, Melhem N, Meyrick S, Moochhala S, Morgan P, Morgan A, Muhammad F, Murray S, Novobritskaya K, Ong ACM, Oni L, Osmaston K, Padmanabhan N, Parkes S, Patrick J, Pattison J, Paul R, Percival R, Perkins SJ, Persu A, Petchey WG, Pickering MC, Pinney J, Pitcher D, Plumb L, Plummer Z, Popoola J, Post F, Power A, Pratt G, Pusey C, Pywell S, Rabara R, Rabuya M, Raju T, Javier C, Roberts ISD, Roufosse C, Rumjon A, Salama A, Saleem M, Sandford RN, Sandu KS, Sarween N, Sayer JA, Sebire N, Selvaskandan H, Shah S, Sharma A, Sharples EJ, Sheerin N, Shetty H, Shroff R, Simms R, Sinha M, Sinha S, Smith K, Smith L, Srivastava S, Steenkamp R, Stott I, Stroud K, Swallow D, Swift P, Szklarzewicz J, Tam F, Tan K, Taylor R, Tischkowitz M, Thomas K, Tse Y, Turnbull A, Turner AN, Tyerman K, Usher M, Venkat-Raman G, Walker A, Walsh SB, Waters A, Watt A, Webster P, Wechalekar A, Welsh GI, West N, Wheeler D, Wiles K, Willcocks L, Williams A, Williams E, Williams K, Wilson DH, Wilson PD, Winyard P, Wong K, Wood G, Woodward E, Woodward L, Woolf A, Wright D, Wong EKS, Gale DP
Publication type: Article
Publication status: Published
Journal: Kidney International
Year: 2025
Volume: 108
Issue: 3
Pages: 455-469
Print publication date: 01/09/2025
Online publication date: 27/06/2025
Acceptance date: 06/06/2025
Date deposited: 04/09/2025
ISSN (print): 0085-2538
ISSN (electronic): 1523-1755
Publisher: Elsevier BV
URL: https://doi.org/10.1016/j.kint.2025.06.003
DOI: 10.1016/j.kint.2025.06.003
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
PubMed id: 40582408
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