Immunoglobulin A vasculitis nephritis: Current understanding of pathogenesis and treatment

doi:10.5527/wjn.v12.i4.82

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Sep 25, 2023 (publication date) through Mar 5, 2026

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World Journal of Nephrology

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2220-6124

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World J Nephrol. Sep 25, 2023; 12(4): 82-92
Published online Sep 25, 2023. doi: 10.5527/wjn.v12.i4.82

Immunoglobulin A vasculitis nephritis: Current understanding of pathogenesis and treatment

Michela Amatruda, Nicolina Stefania Carucci, Roberto Chimenz, Giovanni Conti

Michela Amatruda, Nicolina Stefania Carucci, Roberto Chimenz, Giovanni Conti, Pediatric Nephrology and Rheumatology Unit, AOU G Martino, University of Messina, Messina 98125, Italy

Author contributions: Amatruda M wrote the article and reviewed the references; Carucci NS wrote the article and revised the references; Chimenz R corrected the article; Conti G thought up the article, supervised the chapters of the article and corrected it.

Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.

Corresponding author: Giovanni Conti, MD, Doctor, Pediatric Nephrology and Rheumatology Unit, AOU G Martino, University of Messina, Via C Valeria 1, Messina 98125, Italy. giovanniconti@hotmail.com

Received: February 16, 2023
Peer-review started: February 16, 2023
First decision: April 13, 2023
Revised: May 16, 2023
Accepted: June 12, 2023
Article in press: June 12, 2023
Published online: September 25, 2023
Processing time: 215 Days and 18 Hours

Abstract

The clinical spectrum of immunoglobulin A vasculitis nephritis (IgAVN) ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome, rapidly progressive glomerulonephritis, or even renal failure. Clinical and experimental studies have shown a multifactor pathogenesis: Infection triggers, impaired glycosylation of IgA1, complement activation, Toll-like-receptor activation and B cell proliferation. This knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations.

Keywords: Immunoglobulin A vasculitis nephritis; Immunoglobulin A vasculitis; Henoch-Schoenlein purpura; Immunoglobulin A nephropathy; Vasculitis, glomerulonephritis

Core Tip: This review summarizes the main mechanisms involved in the pathogenesis of immunoglobulin A vasculitis nephritis and the recent treatment development, in order to decrease the risk of kidney disease progression.