IgA vasculitis with nephritis: update of pathogenesis with clinical implications

Hastings, Margaret; Rizk, Dana V.; Kiryluk, Krzysztof; Nelson, Raoul D.; Zahr, Rima S.; Novák, Jan; Wyatt, Robert

doi:10.1007/s00467-021-04950-y

Cited by 45 publications

(51 citation statements)

References 121 publications

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“…The results reflected that both cellular and humoral immunity disorder participated in the pathogenesis of HSP and HSPN. Although IgA circulating immune complexes deposition plays a key role in HSP, and previous studies suggested HSP is linked with increased serum IgA levels, 21 , 22 in our patients, IgA levels showed no relationship with renal involvement. Recent studies have shown that abnormal coagulation and fibrinolysis participate in the occurrence and development of HSP and HSPN.…”

Section: Discussioncontrasting

confidence: 68%

Predictive Value of Laboratory Indexes on Renal Involvement in Children with Henoch-Schönlein Purpura

Sun

et al. 2021

IJGM

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Section: Discussioncontrasting

confidence: 68%

Predictive Value of Laboratory Indexes on Renal Involvement in Children with Henoch-Schönlein Purpura

Sun

et al. 2021

IJGM

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“…Pathogenesis of both IgAN and IgAVN is thought to occur through a multi-hit process [ 5 ]. This process includes the production of galactose-deficient IgA1 (Gd-IgA1) [ 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 ], generation of circulating IgG autoantibodies specific for Gd-IgA1 [ 29 , 30 , 31 , 32 , 33 , 34 ], formation of pathogenic Gd-IgA1-containing immune complexes [ 30 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 ], and the subsequent mesangial deposition of these immune complexes resulting in glomerular injury [ 34 , 44 ].…”

Section: Discussionmentioning

confidence: 99%

“…Circulating levels of Gd-IgA1 and Gd-IgA1-specific IgG autoantibodies are elevated in patients with IgAVN but not in patients with IgAV [ 29 ], supporting the hypothesis that IgAVN and IgAN share pathogenetic components. IgAVN patients have the onset of disease defined by purpura, with kidney involvement developing with 4–6 weeks later [ 5 , 13 , 17 ]. However, there is a limited information about histopathologic disease severity in relation to disease onset.…”

Section: Discussionmentioning

confidence: 99%

“…A minority of pediatric IgAV patients exhibits kidney involvement—IgAV with nephritis (IgAVN)—usually 4–6 weeks after the onset of purpura [ 2 , 3 , 4 , 5 ]. The kidney-biopsy histopathologic features of IgAVN are similar to those of IgA nephropathy (IgAN), including glomerular IgA1-containing immunodeposits [ 1 , 2 , 4 , 6 , 7 , 8 , 9 , 10 , 11 ].…”

Section: Introductionmentioning

confidence: 99%

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IgA Vasculitis with Nephritis in Adults: Histological and Clinical Assessment

Lai

Liu

Azrad

et al. 2021

JCM

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Patients with IgA vasculitis (IgAV), an immune complex-mediated disease, may exhibit kidney involvement—IgAV with nephritis (IgAVN). The kidney-biopsy histopathologic features of IgAVN are similar to those of IgA nephropathy, but little is known about histopathologic disease severity based on the interval between purpura onset and diagnostic kidney biopsy. We assessed kidney histopathology and clinical and laboratory data in a cohort of adult patients with IgAVN (n = 110). The cases were grouped based on the interval between the onset of purpura and kidney biopsy: Group 1 (G1, <1 month, n = 14), Group 2 (G2, 1–6 months, n = 58), and Group 3 (G3, >6 months, n = 38). Glomerular leukocytes were more common in G1 than in the other groups (p = 0.0008). The proportion of neutrophils among peripheral-blood leukocytes was the highest in the patients biopsied within a month after onset of purpura (G1: 71 ± 8%). In the patients with an interval >6 months, the neutrophil proportion was lower, 60%. Moreover, the glomerular mesangial proliferation score correlated with the serum total IgA concentration (p = 0.0056). In conclusion, IgAVN patients biopsied <1 month from purpura onset showed an elevated percentage of blood neutrophils and glomerular leukocytes, consistent with an acute-onset inflammatory reaction. In all IgAVN patients, the mesangial proliferation score correlated with the serum IgA level.

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“…In IgA1, this unique O-linked carbohydrate site seems to be associated with the pathogenesis of IgA nephritis from the dominant deposition of galactose-deficient IgA compared with IgA vasculitis without nephritis and healthy subjects. Interestingly, there was no significant difference in galactose-deficient IgA1 deposition between healthy subjects and IgA vasculitis without nephritis [ 62 ], suggesting that there is a different IgA1 dominant deposition pattern in the skin and kidneys in patients with IgA vasculitis.…”

Section: Triggers and Pathogenesis Of Iga Vasculitismentioning

confidence: 99%

IgA Vasculitis: Etiology, Treatment, Biomarkers and Epigenetic Changes

Sugino

Sawada

Nakamura

2021

IJMS

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IgA, previously called Henoch-Schönlein vasculitis, is an essential immune component that drives the host immune response to the external environment. As IgA has the unique characteristic of a flexible response to broad types of microorganisms, it sometimes causes an autoreactive response in the host human body. IgA vasculitis and related organ dysfunction are representative IgA-mediated autoimmune diseases; bacterial and viral infections often trigger IgA vasculitis. Recent drug developments and the presence of COVID-19 have revealed that these agents can also trigger IgA vasculitis. These findings provide a novel understanding of the pathogenesis of IgA vasculitis. In this review, we focus on the characteristics of IgA and symptoms of IgA vasculitis and other organ dysfunction. We also mention the therapeutic approach, biomarkers, novel triggers for IgA vasculitis, and epigenetic modifications in patients with IgA vasculitis.

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IgA vasculitis with nephritis: update of pathogenesis with clinical implications

Cited by 45 publications

References 121 publications

Predictive Value of Laboratory Indexes on Renal Involvement in Children with Henoch-Schönlein Purpura

Predictive Value of Laboratory Indexes on Renal Involvement in Children with Henoch-Schönlein Purpura

IgA Vasculitis with Nephritis in Adults: Histological and Clinical Assessment

IgA Vasculitis: Etiology, Treatment, Biomarkers and Epigenetic Changes

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