IgA Nephropathy
A chronic, progressive disease rooted in autoimmunity. IgAN is the most common primary glomerular disease worldwide, with an estimated incidence of 2.5/100k per year in adults. 1
IgAN causes progressive, irreversible kidney damage and can have a significant impact on quality of life and overall life expectancy
- Commonly diagnosed in adults under age 40, with most patients presenting with demonstrable chronic kidney disease at time of diagnosis.2,3
- At least 50% of patients progress to kidney failure within 10 years of diagnosis, and the remaining 50% remain at high risk for the rest of their lives.3
- Kidney failure requires dialysis or kidney transplantation, which are associated with reduced quality of life and can result in premature death.2
IgAN is a B-cell–mediated autoimmune kidney disease in which Gd-IgA1, an altered pathogenic form of the IgA antibody, and corresponding autoantibodies form immune complexes that deposit in the glomeruli. These deposits cause chronic inflammation and can lead to kidney injury and progressive loss of kidney function over time.3-5
IgAN Pathophysiology6
APRIL, A proliferation-inducing ligand; BAFF, B-cell activating factor, also known as BLyS (B lymphocyte stimulator); Gd-IgA1, galactose-deficient immunoglobulin A1; TACI, transmembrane activator and calcium-modulator and cyclophilin ligand interactor.
References
- 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):414-430. doi:10.1093/ndt/gfq665
- Jarrick S, Lundberg S, Welander A, et al. Mortality in IgA Nephropathy: A Nationwide Population-Based Cohort Study. J Am Soc Nephrol. 2019;30(5):866-876. doi:10.1681/ASN.2018101017
- Kwon CS, Daniele P, Forsythe A, Ngai C. 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. Published 2021 Sep 1. doi:10.36469/001c.26129
- Pitcher D, Braddon F, Hendry B, et al. Long-Term Outcomes in IgA Nephropathy. Clin J Am Soc Nephrol. 2023;18(6):727-738. doi:10.2215/CJN.0000000000000135
- Wyatt RJ, Julian BA. IgA nephropathy. N Engl J Med. 2013;368(25):2402-2414. doi:10.1056/NEJMra1206793
- Cheung CK, Barratt J, Liew A, Zhang H, Tesar V, Lafayette R. The role of BAFF and APRIL in IgA nephropathy: pathogenic mechanisms and targeted therapies. Front Nephrol. 2024;3:1346769. Published 2024 Feb 1. doi:10.3389/fneph.2023.1346769