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

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Journal Front Immunol
Date 2021 Dec 3
PMID 34858429
Citations 47
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Abstract

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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References
1.
Knoppova B, Reily C, Maillard N, Rizk D, Moldoveanu Z, Mestecky J . The Origin and Activities of IgA1-Containing Immune Complexes in IgA Nephropathy. Front Immunol. 2016; 7:117. PMC: 4828451. DOI: 10.3389/fimmu.2016.00117. View

2.
Dolezalova P, Telekesova P, Nemcova D, Hoza J . Incidence of vasculitis in children in the Czech Republic: 2-year prospective epidemiology survey. J Rheumatol. 2004; 31(11):2295-9. View

3.
Aleyd E, Al M, Tuk C, van der Laken C, van Egmond M . IgA Complexes in Plasma and Synovial Fluid of Patients with Rheumatoid Arthritis Induce Neutrophil Extracellular Traps via FcαRI. J Immunol. 2016; 197(12):4552-4559. DOI: 10.4049/jimmunol.1502353. View

4.
Yang Y, Hung C, Hsu C, Wang L, Chuang Y, Lin Y . A nationwide survey on epidemiological characteristics of childhood Henoch-Schönlein purpura in Taiwan. Rheumatology (Oxford). 2005; 44(5):618-22. DOI: 10.1093/rheumatology/keh544. View

5.
Cassol C, Bott C, Nadasdy G, Alberton V, Malvar A, Nagaraja H . Immunostaining for galactose-deficient immunoglobulin A is not specific for primary immunoglobulin A nephropathy. Nephrol Dial Transplant. 2019; 35(12):2123-2129. DOI: 10.1093/ndt/gfz152. View