IgA nephropathy (IgAN) is the commonest pattern of glomerulonephritis worldwide, it accounts for approximately 10% to 50% of biopsy-proven primary glomerular diseases.[10]Schena FP, Nistor I. Epidemiology of IgA nephropathy: a global perspective. Semin Nephrol. 2018 Sep;38(5):435-42.
https://www.doi.org/10.1016/j.semnephrol.2018.05.013
http://www.ncbi.nlm.nih.gov/pubmed/30177015?tool=bestpractice.com
In one systematic review, the estimated annual global incidence of IgAN in adults was 2.5 in 100,000.[11]McGrogan A, Franssen CF, de Vries CS. The incidence of primary glomerulonephritis worldwide: a systematic review of the literature. Nephrol Dial Transplant. 2011 Feb;26(2):414-30.
https://www.doi.org/10.1093/ndt/gfq665
http://www.ncbi.nlm.nih.gov/pubmed/21068142?tool=bestpractice.com
Annual incidence in the US has been estimated at 1.29 in 100,000.[12]Kwon CS, Daniele P, Forsythe A, et al. A systematic literature review of the epidemiology, health-related quality of life impact, and economic burden of Immunoglobulin A nephropathy. J Health Econ Outcomes Res. 2021;8(2):36-45.
https://www.doi.org/10.36469/001c.26129
http://www.ncbi.nlm.nih.gov/pubmed/34692885?tool=bestpractice.com
IgAN affects nearly all ages, but the peak incidence is in the second and third decades of life.[13]Knoppova B, Reily C, King RG, et al. Pathogenesis of IgA nephropathy: current understanding and implications for development of disease-specific treatment. J Clin Med. 2021 Sep 29;10(19):4501.
https://www.doi.org/10.3390/jcm10194501
http://www.ncbi.nlm.nih.gov/pubmed/34640530?tool=bestpractice.com
[14]Penfold RS, Prendecki M, McAdoo S, et al. Primary IgA nephropathy: current challenges and future prospects. Int J Nephrol Renovasc Dis. 2018;11:137-48.
https://www.doi.org/10.2147/IJNRD.S129227
http://www.ncbi.nlm.nih.gov/pubmed/29695925?tool=bestpractice.com
There is regional variability with incidence of IgAN higher in Asian people than people from Europe, the US, or Africa.[11]McGrogan A, Franssen CF, de Vries CS. The incidence of primary glomerulonephritis worldwide: a systematic review of the literature. Nephrol Dial Transplant. 2011 Feb;26(2):414-30.
https://www.doi.org/10.1093/ndt/gfq665
http://www.ncbi.nlm.nih.gov/pubmed/21068142?tool=bestpractice.com
[13]Knoppova B, Reily C, King RG, et al. Pathogenesis of IgA nephropathy: current understanding and implications for development of disease-specific treatment. J Clin Med. 2021 Sep 29;10(19):4501.
https://www.doi.org/10.3390/jcm10194501
http://www.ncbi.nlm.nih.gov/pubmed/34640530?tool=bestpractice.com
These rates may differ, at least in part, due to differences in public health screening and biopsy practices. In countries with active screening programs for kidney disease, and centers with a low threshold to perform a kidney biopsy, the reported incidence of IgAN is higher, as more patients with mild disease are identified. However, autopsy and genetic data also suggest that IgAN may be more common in Asian populations.[15]Rodrigues JC, Haas M, Reich HN. IgA nephropathy. Clin J Am Soc Nephrol. 2017 Apr 3;12(4):677-86.
https://www.doi.org/10.2215/CJN.07420716
http://www.ncbi.nlm.nih.gov/pubmed/28159829?tool=bestpractice.com
A clearly defined west-to-east increase in disease incidence, and distribution of currently understood genetic risk alleles, occurs from North America to Asia. The precise contribution of specific alleles, however, remains unclear.
In the four extensively published familial IgAN kindreds, the genetic linkage differs in each kindred.[16]Gharavi AG, Yan Y, Scolari F, et al. IgA nephropathy, the most common cause of glomerulonephritis, is linked to 6q22-23. Nat Genet. 2000 Nov;26(3):354-7.
http://www.ncbi.nlm.nih.gov/pubmed/11062479?tool=bestpractice.com
[17]Bisceglia L, Cerullo G, Forabosco P, et al. Genetic heterogeneity in Italian families with IgA nephropathy: suggestive linkage for two novel IgA nephropathy loci. Am J Hum Genet. 2006 Dec;79(6):1130-4.
http://www.cell.com/ajhg/fulltext/S0002-9297(07)63477-6
http://www.ncbi.nlm.nih.gov/pubmed/17186473?tool=bestpractice.com
[18]Paterson AD, Liu XQ, Wang K, et al. Genome-wide linkage scan of a large family with IgA nephropathy localizes a novel susceptibility locus to chromosome 2q36. J Am Soc Nephrol. 2007 Aug;18(8):2408-15.
http://jasn.asnjournals.org/content/18/8/2408.long
http://www.ncbi.nlm.nih.gov/pubmed/17634434?tool=bestpractice.com
[19]Karnib HH, Sanna-Cherchi S, Zalloua PA, et al. Characterization of a large Lebanese family segregating IgA nephropathy. Nephrol Dial Transplant. 2007 Mar;22(3):772-7.
https://academic.oup.com/ndt/article/22/3/772/1899254
http://www.ncbi.nlm.nih.gov/pubmed/17172253?tool=bestpractice.com
Genome-wide analysis has identified several genetic susceptibility loci, but these do not map to the loci identified in the published familial IgAN kindreds.[20]Kiryluk K, Li Y, Sanna-Cherchi S, et al. Geographic differences in genetic susceptibility to IgA nephropathy: GWAS replication study and geospatial risk analysis. PLoS Genet. 2012;8(6):e1002765.
http://journals.plos.org/plosgenetics/article?id=10.1371/journal.pgen.1002765
http://www.ncbi.nlm.nih.gov/pubmed/22737082?tool=bestpractice.com
[21]Gharavi AG, Kiryluk K, Choi M, et al. Genome-wide association study identifies susceptibility loci for IgA nephropathy. Nat Genet. 2011 Mar 13;43(4):321-7.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3412515
http://www.ncbi.nlm.nih.gov/pubmed/21399633?tool=bestpractice.com
[22]Feehally J, Farrall M, Boland A, et al. HLA has strongest association with IgA nephropathy in genome-wide analysis. J Am Soc Nephrol. 2010 Oct;21(10):1791-7.
http://jasn.asnjournals.org/content/21/10/1791.long
http://www.ncbi.nlm.nih.gov/pubmed/20595679?tool=bestpractice.com
[23]Kiryluk K, Li Y, Scolari F, et al. Discovery of new risk loci for IgA nephropathy implicates genes involved in immunity against intestinal pathogens. Nat Genet. 2014 Nov;46(11):1187-96.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4213311
http://www.ncbi.nlm.nih.gov/pubmed/25305756?tool=bestpractice.com