Executive summary of the KDIGO 2025 Clinical Practice Guideline for the Management of Immunoglobulin A Nephropathy (IgAN) and Immunoglobulin A Vasculitis (IgAV)

Abstract

The Immunoglobulin A nephropathy (IgAN) and Immunoglobulin A vasculitis (IgAV) management guideline was last updated and published as part of the Kidney Disease: Improving Global Outcomes (KDIGO) 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Based on new developments in IgAN assessment and therapy, a major update of the guideline was necessary in 2024. Compared with the last version, the KDIGO 2025 IgAN guideline now encourages a more liberal kidney biopsy policy and suggests aiming for stricter proteinuria control, with a goal of <0.5 g/d, ideally <0.3 g/d, and a stable estimated glomerular filtration rate. A major new concept in the 2025 guideline is to initiate treatment with (i) therapies that prevent or reduce pathogenic IgA production and IgA/IgA and IgA/IgG immune complex formation along with (ii) therapies to manage the consequences of existing IgAN-induced nephron loss. Approaches to achieve the first aim are currently limited to targeted-release budesonide (Nefecon) or reduced-dose systemic corticosteroid therapy and, in Chinese patients, mycophenolate mofetil. Approaches to the more generic second aim include healthy lifestyle education, renin-angiotensin system blockers, sodium-glucose cotransporter-2 inhibitors, and/or dual endothelin angiotensin receptor blockers. Little has changed for special situations of IgA-dominant immune complex glomerular diseases such as nephrotic syndrome, acute kidney injury, rapidly progressive glomerulonephritis, and pregnancy in IgAN, or children with IgAN or IgAV, given the lack of major clinical trials in these patient populations. Here, we provide an executive summary of the most important changes in the KDIGO 2025 IgAN and IgAV guideline as a quick reference.

Keywords: IgA nephropathy; IgA vasculitis; KDIGO; glomerular diseases; glomerulonephritis; guideline.