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ISSN: 1426-3912
Central European Journal of Immunology
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vol. 43
Clinical immunology

Serum GDIgA1 levels in children with IgA nephropathy and Henoch-Schönlein nephritis

Małgorzata Mizerska-Wasiak, Łukasz Gajewski, Karolina Cichoń-Kawa, Jadwiga Małdyk, Katarzyna Dziedzic-Jankowska, Beata Leszczyńska, Agnieszka Rybi-Szumińska, Anna Wasilewska, Agnieszka Pukajło-Marczyk, Danuta Zwolińska, Beata Bieniaś, Przemysław Sikora, Maria Szczepańska, Anna Stelmaszczyk-Emmel, Elżbieta Górska, Małgorzata Pańczyk-Tomaszewska

(Centr Eur Immunol 2018; 43 (2): 162-167)
Online publish date: 2018/06/30
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GDIgA1 (galactose deficient IgA1) plays a significant role in the pathogenesis of IgA nephropathy (IgAN) and Henoch-Schönlein nephritis (HSN)

Aim and study
The aim of this study was to assess the relevance of serum GDIgA1 level as a prognostic marker in children with IgAN and HSN.

Material and Methods
41 children were included to the study group (15 IgAN, 26 HSN) and 22 to the control group. The following parameters were evaluated at baseline and endpoint: proteinuria, erythrocyturia, serum creatinine, serum IgA, GFR. A kidney biopsy was performed in all patients and evaluated according to the Oxford Classification (1 – present, 0 – absent: M – mesangial hypercellularity; E– endocapillary hypercellularity; S – segmental sclerosis/adhesion; T – tubular atrophy/interstitial fibrosis), and was calculated as the total score (sum of M, E, S, T). At the end of follow-up, the serum GDIgA1 concentration was measured.

The serum GDIgA1 concentration in patients with IgAN and HSN was significantly higher than in the control group. No significant differences in mean proteinuria, erythrocyturia, GFR, MEST score, or GDIgA1 in serum, as well as the duration of follow-up between IgAN and HSN were observed. Baseline serum IgA concentration and time to kidney biopsy were significantly higher in children with IgAN than in children with HSN. We observed a positive correlation between GDIgA1 and IgA levels (r = 0.53), and GDIgA1 and serum creatinine levels (r = 0.5), as well as negative correlation between GDIgA1 and GFR (r = –0.37).

Serum GDIgA1 level may have a prognostic value in children with IgAN and HSN; however, to fully elucidate its clinical potential further studies performed in larger patient cohorts are required.


galactose deficient IgA1, IgAN, HSN, children

Hogg RJ (2010): Idiopathic immunoglobulin A nephropathy in children and adolescents. Pediatr Nephrol 25: 823-829.
Mizerska-Wasiak M (2009): Nefropatia IgA. Nowa Pediatr 2: 50-55.
Nair R, Walker PD (2006): Is IgA nephropathy the commonest primary glomerulopathy among young adults in the USA? Kidney Int 69: 1455-1458.
Trnka P (2013): Henoch-Schonlein purpura in children. J Paediatr Child Health 49: 995-1003.
Piram M, Mahr A (2013): Epidemiology of immunoglobulin A vasculitis (Henoch-Schonlein): current state of knowledge. Curr Opin Rheumatol 25: 171-178.
Lau K, Wyatt R, Moldoveanu Z, et al. (2007): Serum levels of galactose-deficient IgA in children with IgA nephropathy and Henoch-Schonlein purpura. Pediatr Nephrol 22: 2067-2072.
Placzek WJ, Yanagawa H, Makita Y, et al. (2018): Serum galactose-deficient-IgA1 and IgG autoantibodies correlate in patients with IgA nephropathy. PLoS ONE 13: e0190967.
Berthoux F, Suzuki H, Thibaudin L, et al. (2012): Autoanti­bodies Targeting Galactose-Deficient IgA1Associate with Progression of IgA Nephropathy. J Am SocNephrol 23: 1579-1587.
Rauen T, Floege J (2017): Inflammation in IgA nephropathy. Pediatr Nephrol 32: 2215-2224.
Mestecky J, Tomana M, Moldoveanu Z, et al. (2008): Role of Aberrant Glycosylation of IgA1 Molecules in the Pathogenesis of IgA Nephropathy. Kidney Blood Press Res 31: 29-37.
Jennewein MF, Alter G (2017): The Immunoregulatory Roles of Antibody Glycosylation. Trends Immunol 38: 358-372.
Suzuki H, Kiryluk K, Novak J et al. (2011): The pathophysiology of IgA nephropathy. J Am Soc Nephrol 22: 1795-1803.
Heineke MH, Ballering AV, Jamin A, et al. (2017): New insights in the pathogenesis of immunoglobulin Avasculitis (Henoch-Schönleinpurpura). Autoimmun Rev 16: 1246-1253.
Berthelot L, Robert T, Vuiblet V, et al. (2015): Recurrent IgA nephropathy is predicted by altered glycosylated IgA, autoantibodies and soluble CD89 complexes. Kidney Int 88: 815-822.
Suzuki H, Yasutake J, Makita Y, et al. (2018): IgA nephropathy and IgA vasculitis with nephritis have a shared feature involving galactose-deficient IgA1-oriented pathogenesis. Kidney Int 93: 700-705.
Kiryluk K, Moldoveanu Z, Sanders JT, et al. (2011): Aberrant glycosylation of IgA1 is inherited in both pediatric IgA nephropathy and Henoch-Schoenlein purpura nephritis. Kidney Int 80: 79-87.
Pillebout E, Jamin A, Ayari H, et al. (2017): Biomarkers of IgA vasculitis nephritis in children. PLoS ONE 12: e0188718.
Lin X, Ding J, Zhu L, et al. (2009): Aberrant galactosylation of IgA1 is involved in the genetic susceptibility of Chinese patients with IgA nephropathy. Nephrol Dial Transplant 24: 3372-3375.
Kiryluk K, Li Y, Moldoveanu Z, et al. (2017): GWAS for serum galactose-deficient IgA1 implicates critical genes of the O-glycosylation pathway. PLoS Genet 13: e1006609.
Moldoveanu Z, Wyatt RJ, Lee JY, et al. (2007): Patients with IgA nephropathy have increased serum galactose-deficient IgA1 levels. Kidney Int 71: 1148-1154.
Jiang M, Jiang X, Rong L, et al. (2015): Serum galactose-deficient IgA1 levels in children with IgA nephropathy. Int J Clin Exp Med 8: 7861-7866.
Shimozato S, Hiki Y, Odani H, et al. (2008): Serum under-galactosylated IgA1 is increased in Japanese patients with IgA nephropathy. Nephrol Dial Transplant 23: 1931-1939.
Hastings MC, Afshan S, Sanders JT, et al. (2012): Serum Galactose-Deficient IgA1 Level Is Not Associated with Proteinuria in Children with IgA Nephropathy. Int J Nephrol 2012: 31546724.
Zhao N, Hou P, Lv J, Moldoveanu Z, Li Y, Kiryluk K, (2012): The level of galactose-deficient IgA1 in the sera of patients with IgA nephropathy is associated with disease progression. Kidney Int 82: 790-796.
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