McGrogan A, Franssen CF, de Vries CS. The incidence of primary glomerulonephritis worldwide: a systematic review of the literature. Nephrol Dial Transplant. 2011;26(2):414–30.

Article  PubMed  Google Scholar 

Pitcher D, Braddon F, Hendry B, Mercer A, Osmaston K, Saleem MA, et al. Long-term outcomes in IgA nephropathy. Clin J Am Soc Nephrol. 2023;18(6):727–38.

Article  PubMed  PubMed Central  Google Scholar 

Shen X, Chen P, Liu M, Liu L, Shi S, Zhou X, et al. Long-term outcomes of IgA nephropathy in China. Nephrol Dial Transplant. 2025;40(6):1137–46.

Article  PubMed  Google Scholar 

Moriyama T, Tanaka K, Iwasaki C, Oshima Y, Ochi A, Kataoka H, et al. Prognosis in IgA nephropathy: 30-year analysis of 1,012 patients at a single center in Japan. PLoS ONE. 2014;9(3):e91756.

Article  PubMed  PubMed Central  Google Scholar 

U.S. Department of Health and Human Services Food and Drug Administration, Center for Drug Evaluation and Research (CDER), Center for Biologics Evaluation and Research (CBER). Qualification Process for Drug Development Tools: Guidance for Industry and FDA Staff. https://www.fda.gov/media/133511/download. Accessed 14 May 2025.

TARPEYO (budesonide) delayed release capsules, for oral use. https://www.accessdata.fda.gov/drugsatfda_docs/label/2021/215935s000lbl.pdf. Accessed 14 May 2025.

FILSPARI® (sparsentan) tablets, for oral use. https://www.accessdata.fda.gov/drugsatfda_docs/label/2024/216403s003lbl.pdf. Accessed 14 May 2025.

FABHALTA® (iptacopan) capsules, for oral use. https://www.accessdata.fda.gov/drugsatfda_docs/label/2024/218276s001lbl.pdf. Accessed 14 May 2025.

VANRAFIA™ (atrasentan) tablets, for oral use. https://www.accessdata.fda.gov/drugsatfda_docs/label/2025/219208s000lbl.pdf. Accessed 14 May 2025.

Kinpeygo : EPAR - Product Information. https://www.ema.europa.eu/en/documents/product-information/kinpeygo-epar-product-information_en.pdf. Accessed 14 May 2025.

Filspari : EPAR - Product information. https://www.ema.europa.eu/en/documents/product-information/filspari-epar-product-information_en.pdf. Accessed 14 May 2025.

Reich HN, Troyanov S, Scholey JW, Cattran DC, Toronto Glomerulonephritis Registry. Remission of proteinuria improves prognosis in IgA nephropathy. J Am Soc Nephrol. 2007;18(12):3177–83.

Article  CAS  PubMed  Google Scholar 

Geissbühler M, Hincapié CA, Aghlmandi S, Zwahlen M, Jüni P, da Costa BR. Most published meta-regression analyses based on aggregate data suffer from methodological pitfalls: a meta-epidemiological study. BMC Med Res Methodol. 2021;21(1):123.

Article  PubMed  PubMed Central  Google Scholar 

Inker LA, Mondal H, Greene T, Masaschi T, Locatelli F, Schena FP, et al. Early change in urine protein as a surrogate end point in studies of IgA nephropathy: an individual-patient meta-analysis. Am J Kidney Dis. 2016;68(3):392–401.

Article  CAS  PubMed  Google Scholar 

Thompson A, Carroll K, Inker AL, Floege J, Perkovic V, Boyer-Suavet S, et al. Proteinuria reduction as a surrogate end point in trials of IgA nephropathy. Clin J Am Soc Nephrol. 2019;14(3):469–81.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Rauen T, Eitner F, Fitzner C, Sommerer C, Zeier M, Otte B, et al. Intensive supportive care plus immunosuppression in IgA nephropathy. N Engl J Med. 2015;373(23):2225–36.

Article  CAS  PubMed  Google Scholar 

Lv J, Zhang H, Wong MG, Jardine MJ, Hladunewich M, Jha V, et al. Effect of oral methylprednisolone on clinical outcomes in patients with IgA Nephropathy: the TESTING randomized clinical trial. JAMA. 2017;318(5):432–42.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Donadio JV Jr, Grande JP, Bergstralh EJ, Dart RA, Larson TS, Spencer DC. The long-term outcome of patients with IgA nephropathy treated with fish oil in a controlled trial. Mayo Nephrology Collaborative Group. J Am Soc Nephrol. 1999;10(8):1772–7.

Article  CAS  PubMed  Google Scholar 

Donadio JV Jr, Larson TS, Bergstralh EJ, Grande JP. A randomized trial of high-dose compared with low-dose omega-3 fatty acids in severe IgA nephropathy. J Am Soc Nephrol. 2001;12(4):791–9.

Article  CAS  PubMed  Google Scholar 

Praga M, Gutiérrez E, González E, Morales E, Hernández E. Treatment of IgA nephropathy with ACE inhibitors: a randomized and controlled trial. J Am Soc Nephrol. 2003;14(6):1578–83.

Article  CAS  PubMed  Google Scholar 

Li PK, Leung CB, Chow KM, Cheng YL, Fung SK, Mak SK, et al. Hong Kong study using valsartan in IgA nephropathy (HKVIN): a double-blind, randomized, placebo-controlled study. Am J Kidney Dis. 2006;47(5):751–60.

Article  CAS  PubMed  Google Scholar 

Katafuchi R, Ikeda K, Mizumasa T, Tanaka H, Ando T, Yanase T, et al. Controlled, prospective trial of steroid treatment in IgA nephropathy: a limitation of low-dose prednisolone therapy. Am J Kidney Dis. 2003;41(5):972–83.

Article  CAS  PubMed  Google Scholar 

Pozzi C, Andrulli S, Del Vecchio L, Melis P, Fogazzi GB, Altieri P, et al. Corticosteroid effectiveness in IgA nephropathy: long-term results of a randomized, controlled trial. J Am Soc Nephrol. 2004;15(1):157–63.

Article  CAS  PubMed  Google Scholar 

Manno C, Torres DD, Rossini M, Pesce F, Schena FP. Randomized controlled clinical trial of corticosteroids plus ACE-inhibitors with long-term follow-up in proteinuric IgA nephropathy. Nephrol Dial Transplant. 2009;24(12):3694–701.

Article  CAS  PubMed  Google Scholar 

Maes BD, Oyen R, Claes K, Evenepoel P, Kuypers D, Vanwalleghem J, et al. Mycophenolate mofetil in IgA nephropathy: results of a 3-year prospective placebo-controlled randomized study. Kidney Int. 2004;65(5):1842–9.

Article  CAS  PubMed  Google Scholar 

Frisch G, Lin J, Rosenstock J, Markowitz G, D’Agati V, Radhakrishnan J, et al. Mycophenolate mofetil (MMF) vs placebo in patients with moderately advanced IgA nephropathy: a double-blind randomized controlled trial. Nephrol Dial Transplant. 2005;20(10):2139–45.

Article  CAS  PubMed  Google Scholar 

Pozzi C, Andrulli S, Pani A, Scaini P, Del Vecchio L, Fogazzi G, et al. Addition of azathioprine to corticosteroids does not benefit patients with IgA nephropathy. J Am Soc Nephrol. 2010;21(10):1783–90.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Pozzi C, Andrulli S, Pani A, Scaini P, Roccatello D, Fogazzi G, et al. IgA nephropathy with severe chronic renal failure: a randomized controlled trial of corticosteroids and azathioprine. J Nephrol. 2013;26(1):86–93.

Article  CAS  PubMed  Google Scholar 

Heerspink HJL, Radhakrishnan J, Alpers CE, Barratt J, Bieler S, Diva U, et al. Sparsentan in patients with IgA nephropathy: a prespecified interim analysis from a randomised, double-blind, active-controlled clinical trial. Lancet. 2023;401(10388):1584–94.

Article  CAS  PubMed  Google Scholar 

Rovin BH, Barratt J, Heerspink HJL, Alpers CE, Bieler S, Chae DW, et al. Efficacy and safety of sparsentan versus irbesartan in patients with IgA nephropathy (PROTECT): 2-year results from a randomised, active-controlled, phase 3 trial. Lancet. 2023;402(10417):2077–90.

Article  CAS  PubMed  Google Scholar 

Joffe MM, Greene T. Related causal frameworks for surrogate outcomes. Biometrics. 2009;65(2):530–8.

Article  PubMed  Google Scholar 

Centre for Reviews and Dissemination. Systematic reviews: Guidance for Undertaking Reviews in Health Care. York: University of York; 2009. https://www.york.ac.uk/media/crd/Systematic_Reviews.pdf. Accessed 14 May 2025.

National Institute of Health and Care Excellence. The guidelines manual. Process and methods (PMG6). https://www.nice.org.uk/process/pmg6/chapter/developing-review-questions-and-planning-the-systematic-review. Accessed 14 May 2025.

Wheeler DC, Toto RD, Stefánsson BV, Jongs N, Chertow GM, Greene T, et al. A pre-specified analysis of the DAPA-CKD trial demonstrates the effects of dapagliflozin on major adverse kidney events in patients with IgA nephropathy. Kidney Int. 2021;100(1):215–24.

Article  CAS  PubMed  Google Scholar 

Hou FF, Xie D, Wang J, Xu X, Yang X, Ai J, et al. Effectiveness of mycophenolate mofetil among patients with progressive IgA nephropathy: a randomized clinical trial. JAMA Netw Open. 2023;6(2):e2254054.