Berthoux FC, Mohey H, Afiani A. Natural history of primary IgA nephropathy. Semin Nephrol. 2008;28:4–9.

Article  PubMed  Google Scholar 

Moriyama T, Tanaka K, Iwasaki C, Oshima Y, Ochi A, Kataoka H, Itabashi M, et al. Prognosis in IgA nephropathy: 30-year analysis of 1012 patients at a single center in Japan. PLoS ONE. 2014;9: e91756.

Article  PubMed  PubMed Central  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  Google Scholar 

Knoop T, Vikse BE, Mwakimonga A, Leh S, Bjørneklett R. Long-term outcome in 145 patients with assumed benign immunoglobulin A nephropathy. Nephrol Dial Transplant. 2017;32(11):1841–50.

Article  CAS  PubMed  Google Scholar 

Kunter U, Floege J. The longer the better: follow-up in seemingly “benign” immunoglobulin A nephropathy. Nephrol Dial Transplant. 2017;32(11):1777–9.

Article  PubMed  Google Scholar 

Yeo SC, Goh SM, Barratt J. Is immunoglobulin A nephropathy different in different ethnic populations? Nephrology. 2019;24:885–95.

Article  PubMed  Google Scholar 

Lee M, Suzuki H, Nihei Y, Matsuzaki K, Suzuki Y. Ethnicity and IgA nephropathy: worldwide differences in epidemiology, timing of diagnosis, clinical manifestations, management and prognosis. Clin Kidney J. 2023;16(Suppl 2):ii1–8.

Article  PubMed  PubMed Central  Google Scholar 

Coppo R, Troyanov S, Bellur S, Cattran D, Cook HT, Feehally J, et al. Validation of the Oxford classification of IgA nephropathy in cohorts with different presentations and treatments. Kidney Int. 2014;86(4):828–36.

Article  CAS  PubMed  PubMed Central  Google Scholar 

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

Article  CAS  PubMed  Google Scholar 

El Karoui K, Fervenza FC, De Vriese AS. Treatment of IgA nephropathy: a rapidly evolving field. J Am Soc Nephrol. 2024;35(1):103–16.

Article  PubMed  Google Scholar 

He P, Wang H, Huang C, He L. Hematuria was a high risk for renal progression and ESRD in immunoglobulin a nephropathy: a systematic review and meta-analysis. Ren Fail. 2021;43(1):488–99.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Working Group of the International IgA Nephropathy Network and the Renal Pathology Society, Cattran DC, Coppo R, Cook HT, Feehally J, Roberts IS, Troyanov S, et al. The Oxford classification of IgA nephropathy: rationale, clinicopathological correlations, and classification. Kidney Int. 2009;76(5):534–45.

Article  Google Scholar 

Barbour SJ, Coppo R, Zhang H, Liu ZH, Suzuki Y, Matsuzaki K, International IgA Nephropathy Network, et al. Evaluating a new international risk-prediction tool in IgA nephropathy. JAMA Intern Med. 2019;179(7):942–52.

Article  PubMed  PubMed Central  Google Scholar 

Barbour SJ, Canney M, Coppo R, Zhang H, Liu ZH, Suzuki Y, International IgA Nephropathy Network, et al. Improving treatment decisions using personalized risk assessment from the International IgA Nephropathy Prediction Tool. Kidney Int. 2020;98(4):1009–19.

Article  CAS  PubMed  Google Scholar 

FDA-NIH Biomarker Working Group: BEST (Biomarkers, EndpointS, and other Tools) resource [Internet]. Silver Spring: Food and Drug Administration (US); 2016. Available at: http://www.ncbi.nlm.nih.gov/books/NBK326791/. Accessed 24 Feb 2018.

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 

Lv J, Wong MG, Hladunewich MA, Jha V, Hooi LS, Monaghan H, TESTING Study Group, et al. Effect of oral methylprednisolone on decline in kidney function or kidney failure in patients with IgA nephropathy: the TESTING randomized clinical trial. JAMA. 2022;327(19):1888–98.

Article  CAS  PubMed  PubMed Central  Google Scholar 

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

Article  CAS  PubMed  Google Scholar 

Thompson A, Carroll K, Inker LA, 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 

Inker LA, Heerspink HJL, Tighiouart H, Chaudhari J, Miao S, Diva U, et al. Association of treatment effects on early change in urine protein and treatment effects on GFR slope in IgA nephropathy: an individual participant meta-analysis. Am J Kidney Dis. 2021;78(3):340-349.e1.

Article  CAS  PubMed  PubMed Central  Google Scholar 

https://www.fda.gov/drugs/nda-and-bla-approvals/accelerated-approval-program. Last viewed on the 6th of December, 2023.

https://www.ema.europa.eu/en/human-regulatory-overview/marketing-authorisation/conditional-marketing-authorisation. Last viewed on the 6th of December, 2023.

Rajasekaran A, Julian BA, Rizk DV. IgA Nephropathy: an interesting autoimmune kidney disease. Am J Med Sci. 2021;361(2):176–94.

Article  PubMed  Google Scholar 

Kiryluk K, Li Y, Moldoveanu Z, Suzuki H, Reily C, Hou P, Xie J, et al. GWAS for serum galactose-deficient IgA1 implicates critical genes of the O-glycosylation pathway. PLoS Genet. 2017;13(2): e1006609.

Article  PubMed  PubMed Central  Google Scholar 

Maixnerova D, Ling C, Hall S, Reily C, Brown R, Neprasova M, et al. Galactose-deficient IgA1 and the corresponding IgG autoantibodies predict IgA nephropathy progression. PLoS ONE. 2019;14(2): e0212254.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Pattrapornpisut P, Avila-Casado C, Reich HN. IgA nephropathy: core curriculum 2021. Am J Kidney Dis. 2021;78(3):429–41.

Article  CAS  PubMed  Google Scholar 

Moldoveanu Z, Suzuki H, Reily C, Satake K, Novak L, Xu N, et al. Experimental evidence of pathogenic role of IgG autoantibodies in IgA nephropathy. J Autoimmun. 2021;118: 102593.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Kiryluk K, Sanchez-Rodriguez E, Zhou XJ, Zanoni F, Liu L, Mladkova N, et al. Genome-wide association analyses define pathogenic signaling pathways and prioritize drug targets for IgA nephropathy. Nat Genet. 2023;55(7):1091–105.

Article  CAS  PubMed  Google Scholar 

Peters B, Beige J, Siwy J, Rudnicki M, Wendt R, Ortiz A, PersTIgAN Working Group, et al. Dynamics of urine proteomics biomarker and disease progression in patients with IgA nephropathy. Nephrol Dial Transplant. 2023;38(12):2826–34.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Monteiro RC, Suzuki Y. Are there animal models of IgA nephropathy? Semin Immunopathol. 2021;43(5):639–48.

Article  CAS  PubMed  Google Scholar 

Rivedal M, Mikkelsen H, Marti HP, Liu L, Kiryluk K, Knoop T, et al. Glomerular transcriptomics predicts long term outcome and identifies therapeutic strategies for patients with assumed benign IgA nephropathy. Kidney Int. 2024;105(4):717–30.

Article  CAS  PubMed  Google Scholar 

Kano T, Suzuki H, Makita Y, Nihei Y, Fukao Y, Nakayama M, et al. Mucosal immune system dysregulation in the pathogenesis of IgA nephropathy. Biomedicines. 2022;10(12):3027.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Hu X, Du J, Xie Y, Huang Q, Xiao Y, Chen J, et al. Fecal microbiota characteristics of Chinese patients with primary IgA nephropathy: a cross-sectional study. BMC Nephrol. 2021;21(1):97.

Article  CAS  Google Scholar 

De Angelis M, Montemurno E, Piccolo M, Vannini L, Lauriero G, Maranzano V, et al. Microbiota and metabolome associated with immunoglobulin A nephropathy (IgAN). PLoS ONE. 2014;9(6): e99006.

Article  PubMed  PubMed Central  Google Scholar 

McCarthy DD, Kujawa J, Wilson C, Papandile A, Poreci U, Porfilio EA, et al. Mice overexpressing BAFF develop a commensal flora-dependent, IgA-associated nephropathy. J Clin Invest. 2011;121(10):3991–4002.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Yeo SC, Barratt J. The contribution of a proliferation-inducing ligand (APRIL) and other TNF superfamily members in pathogenesis and progression of IgA nephropathy. Clin Kidney J. 2023;16(Suppl 2):ii9–18.

Article  PubMed  PubMed Central  Google Scholar 

Mathur M, Chan TM, Oh KH, Kooienga L, Zhuo M, Pinto CS, et al. A PRoliferation-Inducing Ligand (APRIL) in the pathogenesis of immunoglobulin A nephropathy: a review of the evidence. J Clin Med. 2023;12(21):6927.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Zheng N, Wang D, Ming H, Zhang H, Yu X. BAFF promotes proliferatio