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Cited by 329

The DOI matches its Crossref record; 61 of the 61 references it cites were found in a registry or an index.

New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura)

Open access
Heineke, Marieke H., Ballering, Aranka V., Jamin, Agnès, Ben Mkaddem, Sanae, Monteiro, Renato C., Van Egmond, Marjolein·2017·Autoimmunity Reviews

Abstract

Immunoglobulin A vasculitis (IgAV), also referred to as Henoch-Schönlein purpura, is the most common form of childhood vasculitis. The pathogenesis of IgAV is still largely unknown. The disease is characterized by IgA1-immune deposits, complement factors and neutrophil infiltration, which is accompanied with vascular inflammation. Incidence of IgAV is twice as high during fall and winter, suggesting an environmental trigger associated to climate. Symptoms can resolve without intervention, but some patients develop glomerulonephritis with features similar to IgA nephropathy that include hematuria, proteinuria and IgA deposition in the glomerulus. Ultimately, this can lead to end-stage renal disease. In IgA nephropathy immune complexes containing galactose-deficient (Gd-)IgA1 are found and thought to play a role in pathogenesis. Although Gd-IgA1 complexes are also present in patients with IgAV with nephritis, their role in IgAV is disputed. Alternatively, it has been proposed that in IgAV IgA1 antibodies are generated against endothelial cells. We anticipate that such IgA complexes can activate neutrophils via the IgA Fc receptor FcαRI (CD89), thereby inducing neutrophil migration and activation, which ultimately causes tissue damage in IgAV. In this Review, we discuss the putative role of IgA, IgA receptors, neutrophils and other factors such as infections, genetics and the complement system in the pathogenesis of IgA vasculitis.

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Metadata

Authors
Heineke, M. H., Ballering, A. V., Jamin, A., Ben Mkaddem, S., Monteiro, R. C., & Van Egmond, M.
Venue
Autoimmunity Reviews
Publisher
Elsevier BV
Published
2017-12
Volume
16
Issue
12
Pages
1246-1253
ISSN
1568-9972 (ISSN-L)
OpenAlex
W2763037910
Cited by
329
References
61
FWCI
18.43
Type
journal article
Language
en
Open access
hybrid

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CoChat. (2026). Citation check: New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura). https://app.cochat.ai/papers/10.1016/j.autrev.2017.10.009
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Heineke, M. H., Ballering, A. V., Jamin, A., Ben Mkaddem, S., Monteiro, R. C., & Van Egmond, M. (2017). New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura). Autoimmunity Reviews. https://doi.org/10.1016/j.autrev.2017.10.009

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