Association of kidney biopsy findings with short- and medium-term outcomes in children with moderate-to-severe IgA vasculitis nephritis

Clavé, Stéphanie; Sordet, Maud; Tsimaratos, Michel; Decramer, Stéphane; Fila, Marc; Guigonis, Vincent; Faudeux, Camille; Daniel, Laurent; Torrents, Julia; Harambat, Jérôme; Rousset‐Rouvière, Caroline

doi:10.1007/s00431-021-04065-4

Cited by 8 publications

(5 citation statements)

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“…Because IgAV-N and IgAN have similar pathological mechanisms and pathological features [ 2 ], previous studies have tested the application of the MEST-C score in IgAV-N [ 7–9 , 11 , 12 , 16 ]. In this study, we also observed the role of the MEST-C score in the clinical features, pathological changes, and prognosis of IgAV-N.…”

Section: Resultsmentioning

confidence: 99%

“…Recent studies have reported that the MEST-C score is related to clinical features in children with IgAV-N [ 11 , 12 , 16 ], but its application in adults is limited. A few studies in adults found a better correlation between E/T and the prognosis of IgAV-N [ 8 , 12 , 16 ], while in pediatric patients, the MEST-C score (especially S and T) is meaningful in predicting prognosis [ 7 , 9 , 11 , 16 ]. Thus, we also applied the MEST-C score to evaluate the clinical prognosis of patients with IgAV-N.…”

Section: Discussionmentioning

confidence: 99%

“…The IgA Nephropathy Classification Working Group and Kidney Disease: Improving Global Outcomes (KDIGO) suggested that the MEST-C score might be applicable to the assessment of patients with IgAV-N [ 14 , 15 ]. The MEST-C score was not yet sufficient to be used in IgAV-N, particularly in adult patients; thus, its value in IgAV-N remains to be further explored [ 7–9 , 11 , 12 , 16 ]. Furthermore, the ISKDC and MEST-C classifications lack the description of glomerular Bowman’s capsule rupture (BCR), a common renal pathological change in heavy immune-mediated glomerulonephritis [ 17–22 ]; therefore, the effect of BCR on IgAV-N also needs to be addressed, especially in adult patients with severe renal lesions.…”

Section: Introductionmentioning

confidence: 99%

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Bowman’s capsule rupture and the Oxford MEST-C score in adult patients with Immunoglobulin a vasculitis nephritis: a single center experience

Zhao,

Duan,

Chen

et al. 2023

Annals of Medicine

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Objective Bowman’s capsule rupture (BCR) is a glomerular pathological change, but it is still not well recognized in immunoglobulin A vasculitis nephritis (IgAV-N). The Oxford MEST-C score is a classification for IgA nephropathy; however, its clinical correlation and prognostic value in adult patients with IgAV-N are unclear. Methods A retrospective study of 145 adult patients with IgAV-N diagnosed by renal biopsy was conducted. Clinical manifestations, pathological changes and the prognosis of IgAV-N patients were compared depending on the presence or absence of BCR, International Study of Kidney Disease in Children (ISKDC) classification and MEST-C score. The primary endpoint events were end-stage renal disease, renal replacement therapy and all-cause death. Results In total, 51 of 145 (35.17%) patients with IgAV-N presented with BCR. Patients with BCR had more proteinuria, lower serum albumin, and more crescents. Compared with IgAV-N patients with crescents only, 51/100 patients with crescents combined with BCR had a higher proportion of crescents in all glomeruli (15.79% vs. 9.09%; p = 0.003). Patients with higher ISKDC grades had more severe clinical presentation, but it did not reflect the prognosis. However, the MEST-C score not only reflected clinical manifestations but also predicted prognosis ( p < 0.05). BCR contributed to the effectiveness of the MEST-C score in predicting the prognosis of IgAV-N (C-index: 0.845 to 0.855). Conclusions BCR is associated with clinical manifestations and pathological changes in patients with IgAV-N. The ISKDC classification and MEST-C score are related to the patient’s condition, but only the MEST-C score is correlated with the prognosis of patients with IgAV-N, while BCR can improve its predictive ability.

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Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Bowman’s capsule rupture and the Oxford MEST-C score in adult patients with Immunoglobulin a vasculitis nephritis: a single center experience

Zhao,

Duan,

Chen

et al. 2023

Annals of Medicine

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“…The impact of pathological changes on the prognosis of IgAVN has been the focus of clinical research and an important reference indicator for clinical treatment. Clavé et al (21) studied 170 children with biopsy-proven IgAVN diagnosed between 2007 and 2017, with patients with NS at presentation and those with cellular crescents and chronic lesions at initial renal biopsy being more likely to develop renal damage, highlighting the relationship between clinical and histological manifestations of IgAVN and factors affecting renal regression. In a retrospective study published in 2021, Wang et al discussed the predictive value of ISKDC classification and MEST-C scores on the prognosis of 877 children with IgAVN and found that S and T could be used to predict renal prognosis (22).…”

Section: Discussionmentioning

confidence: 99%

Association of the clinicopathological characteristics and proteinuria remission of pediatric IgAV with nephrotic-range proteinuria: A retrospective cohort study

Kang

Xun²,

Li³

et al. 2022

Front. Pediatr.

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BackgroundIgA vasculitis (IgAV) combined with nephrotic-range proteinuria is uncommon, and nephrotic-range proteinuria is considered a risk factor for poor prognosis in children with IgAV. There are few clinical studies with large samples.MethodsChildren with IgAV and nephrotic-range proteinuria who were hospitalized at the Department of Nephrology, Rheumatology and Immunology, Hunan Children's Hospital, from March 2008 to January 2020 were retrospectively studied; the patients were aged ≤18 years and were followed up for ≥12 months. We analyzed clinical characteristics, pathological changes, treatment responses, and their relationships in patients with IgAV combined with nephrotic-range proteinuria.ResultsTwo hundred seventy-seven children with an average age at onset of IgAV with nephritis (IgAVN) of 8.0 years (interquartile range (IQR), 6.0–10.0) were enrolled; 65.7% were aged 6–10 years. The male-to-female ratio was 1.35:1. All children had both nephrotic-range proteinuria and hematuria, 49 (17.7%) had hypoalbuminemia, and 9 (3.2%) had estimated glomerular filtration rate < 90 (mL/min/1.73 m2). All included children were followed up for at least 1 year. At 3, 6, and 12 months of follow-up, the remission rates of proteinuria in children with IgAV combined with nephrotic-range proteinuria were 27.8%, 62.1%, and 83.0%, respectively, and the remission rates of hematuria were 1.4%, 8.7%, and 35.7%, respectively. In addition, children with age at onset of IgAV with nephrotic-range proteinuria ≥10 years, who were female, who had proteinuria ≥100 mg/kg/24 h, and who had a pathological grade III or above had lower remission rates of hematuria and proteinuria (P < 0.05). Multivariate factor analysis was performed by logistic regression and showed age at onset of IgAVN ≥ 10 years and crescents to be risk factors for nonremission of proteinuria at 12 months of follow-up (P < 0.05).ConclusionsAge at onset of IgAVN, sex, proteinuria level, pathological grade, and crescents significantly affect proteinuria remission in children with IgAV combined with nephrotic-range proteinuria.

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“…Aging negatively impacts the severity and outcome of the disease in adult patients with IgAV (12). Younger patients are more frequently involved with the joint and gastrointestinal tract, whereas old patients are at increased risk of severe purpura and glomerulonephritis, including endstage kidney disease (ESKD) (12)(13)(14)(15)(16).…”

Section: Introductionmentioning

confidence: 99%

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

Song

Huang

et al. 2021

Front. Immunol.

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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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Association of kidney biopsy findings with short- and medium-term outcomes in children with moderate-to-severe IgA vasculitis nephritis

Cited by 8 publications

References 39 publications

Bowman’s capsule rupture and the Oxford MEST-C score in adult patients with Immunoglobulin a vasculitis nephritis: a single center experience

Bowman’s capsule rupture and the Oxford MEST-C score in adult patients with Immunoglobulin a vasculitis nephritis: a single center experience

Association of the clinicopathological characteristics and proteinuria remission of pediatric IgAV with nephrotic-range proteinuria: A retrospective cohort study

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

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