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Published in: Pediatric Nephrology 12/2017

01-12-2017 | Educational Review

Inflammation in IgA nephropathy

Authors: Thomas Rauen, Jürgen Floege

Published in: Pediatric Nephrology | Issue 12/2017

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Abstract

Immunoglobulin A nephropathy (IgAN) is the most frequently occurring primary glomerulonephritis in Caucasian and Asian populations. Nonetheless, therapeutic recommendations are based on weak evidence, large controlled trials are scarce and, in particular, the additional value of immunosuppression beyond comprehensive supportive measures is not well-established. The use of immunosuppressants is supported by experimental insights into IgAN pathogenesis that suggest an autoimmune component in disease development. The so-called “multi-hit” theory comprises multiple steps, starting with defective glycosylation of IgA subclass IgA1 that results in overproduction of galactose-deficient IgA1 (Gd-IgA1), occurrence of anti-Gd-IgA1 autoantibodies, and mesangial deposition of nephritogenic immune complexes. This eventually results in an increased mesangial cell proliferation, inflammatory responses, and complement activation. Recent genome-wide association studies have identified several susceptibility genes, many of which support the “multi-hit” concept. In light of these discoveries, it is astonishing that the vast majority of adult IgAN patients obviously do not need and/or benefit from immunosuppressive therapies in the first place. In fact, a number of supportive measures are highly effective in reducing the risk for disease progression in many patients. These measures need to be optimized before immunosuppression should be considered at all. In this review we focus on the underlying pathogenetic cornerstones and the central question of whether systemic inflammation in adult IgAN patients should be treated. Treatment options in children with IgAN are also discussed.
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Metadata
Title
Inflammation in IgA nephropathy
Authors
Thomas Rauen
Jürgen Floege
Publication date
01-12-2017
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 12/2017
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-017-3628-1

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