Presentation of pediatric Henoch–Schönlein purpura nephritis changes with age and renal histology depends on biopsy timing

Hennies, Imke; Gimpel, Charlotte; Gellermann, Jutta; Möller, Kristina; Mayer, Brigitte; Dittrich, Katalin; Büscher, Anja; Hansen, Matthias; Aulbert, Wiebke; Wühl, Elke; Nissel, Richard; Schalk, Gessa; Weber, Lutz T.; Pohl, Michael; Wygoda, Simone; Beetz, Rolf; Klaus, Günter; Fehrenbach, Henry; König, Sabine; Staude, Hagen; Beringer, Ortraud; Bald, Martin; Walden, Ulrike; Schnakenburg, Christian von; Bertram, Gunhard; Wallot, M.; Häffner, Karsten; Wiech, Thorsten; Hoyer, Peter F.; Pöhl, Martin

doi:10.1007/s00467-017-3794-1

Cited by 30 publications

(19 citation statements)

References 31 publications

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“…In our study, like in another German study concerning IgAVN histological features [16], a longer delay between nephritis and kidney biopsy was related with a higher proportion of chronic lesions. Not surprisingly, longer delay after initial inflammation produces more sclerosis or fibrosis.…”

Section: Discussionsupporting

confidence: 86%

“…About one third of their patients had chronic lesions while only 15% of them had EP [20] and Inagaki et al studied the biopsy of 74 patients with IgAVN: GS was present in 50% of patients [21]. Even in children, the difference of histological features depending on patient age was highlighted: These authors found that younger patients had less often GS or chronic lesions on their initial biopsy [16]. The higher proportion of chronic lesions in older patients was also found in a study of IgAN in children [22].…”

Section: Discussionmentioning

confidence: 99%

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Histological prognostic factors in children with Henoch-Schönlein purpura nephritis

et al. 2019

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Introduction. The management of IgA vasculitis with nephritis (IgAVN) remains controversial because of the difficulty to identify prognostic factors. This study reports the prognosis of children with IgAVN in relation to histological parameters. Methods. All children with IgAVN diagnosed between 2000 and 2015 in three pediatric nephrology centers were included. The following histological parameters were analyzed: mesangial proliferation (MP), endocapillary proliferation (EP), crescents, active or chronic tubular and interstitial lesions (TIa lesions / TIc lesions), and segmental glomerulosclerosis (GS). Clinical and biological data were collected at the time of renal biopsy. The primary endpoint was IgAVN remission defined as a proteinuria <200 mg/L without renal failure. Results. 159 children were included with a median age of 7.6 years. Acute glomerular or TI lesions including MP, EP, crescents, and TIa lesions were observed, respectively, in 81%, 86%, 49%, and 21% of patients. Chronic glomerular lesions including GS and TIc lesions were observed in 6 and 7% of patients. Median initial proteinuria was 330 mg/mmol, albuminemia 32 g/l, and eGFR 110 ml/min/1.73m 2. 112 (70%) patients were in remission at the end of a median follow up of 37.4 months. Chronic lesions were significantly associated with the absence of remission in multivariate analysis, whereas EP, crescents and TIa were not associated with a poor prognosis. Conclusion. 30% of children with IgAVN present a persistent renal disease at the end of a 3year follow-up. Chronic histological lesions, but not EP or crescents are associated with a bad prognosis and must be evaluated in IgAVN histological classification.

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Section: Discussionsupporting

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Histological prognostic factors in children with Henoch-Schönlein purpura nephritis

et al. 2019

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“…In patients with chronic lesions, the median time from IgAV-N diagnosis to kidney biopsy was twice as long as that in patients without chronic lesions (30 vs. 17 days). These results were similar to those in the studies by Hennies et al (23) and Delbet et al (27), which reported a longer delay between nephritis and kidney biopsy and a higher proportion of chronic lesions among children with worse outcomes. We also found that the presence of chronic lesions was signi cantly associated with a poorer kidney outcome, as other studies did (32).…”

Section: Discussionsupporting

confidence: 91%

“…The demographic characteristics of our population are similar to those in other studies, with a predominance of male patients and a median onset age of 8 years (11,13,23,24). In accordance with previous studies, kidney involvement occurred within 6 months following IgAV diagnosis in almost all our patients (96%) (4,23,25).…”

Section: Discussionsupporting

confidence: 90%

Association of Kidney Biopsy Findings With Short- and Medium-Term Outcomes in Children With Moderate-to-Severe Iga Vasculitis Nephritis

Clavé

Sordet

Tsimaratos

et al. 2021

Preprint

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Assessing the initial severity of immunoglobulin A vasculitis nephritis (IgAV-N) is challenging important due to its determining effect on kidney management and outcomes. This study paper aims to describe describes a multicentre paediatric multicenter pediatric cohort of IgAV-N patients and discusses whilst investigating the relationships among between clinical presentation, histological features, and kidney outcome. A cohort consisting of 170 children requiring early kidney with biopsy because of IgAV-N, which was diagnosed between 2007 and 2017, was assessed including 27% of children with nephrotic syndrome (NS). One-quarter of the cohort (27%) presented with initial nephrotic syndrome (NS). Kidney biopsy revealed International Study of Kidney Disease (ISKDC) grade II or grade III in 83% of cases. An International Study of Kidney Disease (ISKDC) grade II or grade III was revealed through kidney biopsy in 83% of cases. Endocapillary proliferation was were observed in 73% of patients, and chronic lesions were observed in 25%of patients. Data analysis demonstrated showed a significant association between clinical severity (NS at onset and histological findings such asendocapillary proliferation and cellular crescents. After a median follow-up of 21 months (IQR 12-39), 30% of patients had persistent kidney impairment (proteinuria or decreased eGFR. WorseAt the end of follow-up, kidney outcome impairment was significantly associated more often observed in patients with NS at onset and those with cellular crescents and chronic lesions on initial kidney biopsy.Conclusions: This study highlights the relationship between the clinical and histological presentation of IgAV-N and the factors that affect kidney outcome. The ISKDC classification may be improved by including lesions that are more discriminating for disease severity and prognosis.

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“…HSPN is a major public health problem that accounts for 78.9% of secondary glomerulopathies in pediatric patients (21). Etiologically, persistent purpura or relapse, severe abdominal symptoms (abdominal pain, gastrointestinal bleeding and severe bowel angina), arthritis and being aged >10 years old are the most significant risk factors for pediatric HSPN (22–24). Initially, lncRNAs were assumed to simply be leaky transcription noise (25).…”

Section: Discussionmentioning

confidence: 99%

Differential expression of long non‑coding RNA and mRNA in children with Henoch‑Sch�nlein purpura nephritis

Pang

Wang

et al. 2018

Exp Ther Med

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Long non-coding RNAs (lncRNAs) serve an essential role in regulating immunological functions. However, their impact on Henoch-Schönlein purpura nephritis (HSPN), has remained elusive. The present study determined the expression of lncRNAs and mRNAs in the peripheral blood of 6 children with HSPN and recruited 4 healthy children for comparative study. High-throughput sequencing revealed outstanding differences in lncRNA and mRNA expression, which were verified through reverse transcription-quantitative polymerase chain reaction. Gene Ontology and Kyoto Encyclopedia of Genes and Genomes pathway analyses were used to investigate the associated biological functions and possible mechanisms of lncRNAs and mRNAs in HSPN. A total of 820 differentially expressed lncRNAs between the two groups were identified, of which 34 were upregulated and 786 were downregulated. Simultaneously, a total of 3,557 mRNAs were also identified to be differentially expressed, of which 1,232 were upregulated and 2,325 were downregulated. The results revealed that the expression of lncRNAs including ENST00000378432, ENST00000571370, uc001kfc.1 and uc010qna.2 was decreased in HSPN patients compared with that in healthy controls. These lncRNAs were associated with the p53 signaling pathway and apoptosis-associated genes (AKT2, tumor protein 53, phosphatase and tensin homolog and FAS). Further studies of those lncRNAs will be performed to elucidate their functions in apoptosis. Complete raw data files were deposited in the Gene Expression Omnibus (GEO) at National Center for Biotechnology information under the GEO accession no. GSE102114 ().

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Presentation of pediatric Henoch–Schönlein purpura nephritis changes with age and renal histology depends on biopsy timing

Cited by 30 publications

References 31 publications

Histological prognostic factors in children with Henoch-Schönlein purpura nephritis

Histological prognostic factors in children with Henoch-Schönlein purpura nephritis

Association of Kidney Biopsy Findings With Short- and Medium-Term Outcomes in Children With Moderate-to-Severe Iga Vasculitis Nephritis

Differential expression of long non‑coding RNA and mRNA in children with Henoch‑Sch�nlein purpura nephritis

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