Pathogenesis of IgA Vasculitis: An Up-To-Date Review

Immunoglobin A (IgA) vasculitis (IgAV), formerly called the Henoch-Schönlein purpura (HSP), is a small vessel vasculitis, characterized by IgA1-dominant immune deposition at diseased vessel walls. IgAV is the most common form of vasculitis in children; typical symptoms include palpable purpura, arth...

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Published inFrontiers in immunology Vol. 12; p. 771619
Main Authors Song, Yan, Huang, Xiaohan, Yu, Guizhen, Qiao, Jianjun, Cheng, Jun, Wu, Jianyong, Chen, Jianghua
Format Journal Article
LanguageEnglish
Published Switzerland Frontiers Media S.A 09.11.2021
Subjects
Animals
Environment
Humans
IgA
IgA vasculitis
IgA Vasculitis - etiology
IgA Vasculitis - genetics
IgA Vasculitis - immunology
Immunology
kidney
pathogenesis
skin
skin
kidney
pathogenesis
IgA
IgA vasculitis
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Summary:Immunoglobin A (IgA) vasculitis (IgAV), formerly called the Henoch-Schönlein purpura (HSP), is a small vessel vasculitis, characterized by IgA1-dominant immune deposition at diseased vessel walls. IgAV is the most common form of vasculitis in children; typical symptoms include palpable purpura, arthritis or arthralgia, abdominal pain, and hematuria or proteinuria. Galactose-deficient IgA1 is detected in the tissues of the kidney and skin in patients with IgAV; it forms immune complexes leading to subsequent immune reactions and injuries. This report provides the recent advances in the understanding of environmental factors, genetics, abnormal innate and acquired immunity, and the role of galactose-deficient IgA1 immunocomplexes in the pathogenesis of IgAV.
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This article was submitted to Alloimmunity and Transplantation, a section of the journal Frontiers in Immunology
These authors have contributed equally to this work
Edited by: Vladimir Tesar, Charles University, Czechia
Reviewed by: Tadashi Ariga, Hokkaido University, Japan; Rabia Miray Kisla Ekinci, Ministry of Health, Turkey
ISSN:1664-3224
1664-3224
DOI:10.3389/fimmu.2021.771619