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

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Abstract

Assessing the initial severity of immunoglobulin A vasculitis nephritis (IgAV-N) is important due to its determining effect on kidney management and outcomes. This paper describes a multicentre paediatric cohort of IgAV-N patients and discusses relationships among clinical presentation, histological features, and kidney outcome. We retrospectively studied a cohort of 170 children with biopsy-proven IgAV-N, diagnosed between 2007 and 2017. 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. Endocapillary proliferation was observed in 73% of patients, and chronic lesions were observed in 25%. Data analysis showed a significant association between NS at onset and endocapillary proliferation and cellular crescents. After a median follow-up of 21 months (IQR 12–39), 30% of patients had persistent proteinuria or decreased eGFR. At the end of follow-up, kidney impairment was more often observed in patients with NS at onset and those with cellular crescents and chronic lesions on initial kidney biopsy.

Conclusion: 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.

What is Known:

• Nephrotic syndrome (NS) or kidney failure at diagnosis and cellular crescents in more than 50% of the glomeruli are recognized as risk factors for poor kidney outcome in immunoglobulin A vasculitis nephritis (IgAV-N).

• The reference histological classification of the International Study of Kidney Disease in Children (ISKDC) is primarily based on the presence and number of affected glomeruli (mesangial proliferation, cellular crescents). The updated Oxford classification, which emphasizes tubular atrophy and interstitial fibrosis, is also used to group pathological features of IgAV-N. Both classifications have limitations.

What is New:

• Medical treatment should not be postponed in patients with IgAV-N and NS until after biopsy, as NS at diagnosis is associated with initial histological severity and poorer kidney outcome. This proposal needs to be verified in further studies.

• Endocapillary proliferation is associated with the initial severity of IgAV-N at diagnosis, while chronic glomerular changes and interstitial fibrosis are associated with poorer short- and medium-term kidney outcomes.

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Abbreviations

ACEi:

Angiotensin-converting enzyme inhibitor

ARB:

Angiotensin receptor blocker

CKD:

Chronic kidney disease

EULAR:

European League Against Rheumatism

GFR:

Glomerular filtration rate

IgAV:

Immunoglobulin A vasculitis

IgAV-N:

Immunoglobulin A vasculitis nephritis

IQR:

Interquartile range

ISKDC:

International Study of Kidney Disease in Children

NS:

Nephrotic syndrome

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Acknowledgements

The authors thank the patients who participated in the study.

Author information

Authors and Affiliations

  1. Department of Multidisciplinary Pediatrics, Pediatric Nephrology Unit, Assistance Publique des Hôpitaux de Marseille, Marseille, France

    Stéphanie Clavé, Michel Tsimaratos & Caroline Rousset-Rouvière

  2. Department of Pediatrics, Hôpital Pellegrin-Enfants, University Hospital of Bordeaux, Bordeaux, France

    Maud Sordet & Jérôme Harambat

  3. Department of Pediatric Nephrology, Hôpital des Enfants, University Hospital of Toulouse, Toulouse, France

    Stéphane Decramer

  4. Department of Pediatric Nephrology, Hôpital Arnaud-de-Villeneuve, University Hospital of Montpellier, Montpellier, France

    Marc Fila

  5. Department of Pediatrics, Hôpital Mère-Enfant, University Hospital of Limoges, Limoges, France

    Vincent Guigonis

  6. Department of Pediatrics, Hôpital de l’Archet, University Hospital of Nice, Nice, France

    Camille Faudeux

  7. Department of Pathology, Assistance Publique des Hôpitaux de Marseille, Marseille, France

    Laurent Daniel & Julia Torrents

  8. Aix-Marseille Univ, School of Medicine - La Timone Medical Campus, EA 3279 - CEReSS Health Service Research and Quality of Life Center, Marseille, France

    Mohamed Boucekine

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  1. Stéphanie Clavé
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Contributions

S Clavé, M Sordet, M Tsimaratos, C Rousset-Rouvière, and J Harambat contributed to the study conception and design. Material preparation and data collection were performed by S Clavé and M Sordet. L Daniel and J Torrents were involved in the analysis of pathological data. M Boucekine conducted the data analysis. The first draft of the manuscript was written by S Clavé, M Tsimaratos, C Rousset-Rouvière, and J Harambat. All authors commented on previous versions of the manuscript. All authors have revised the manuscript critically for important intellectual content, have provided final approval of the manuscript version to be published, and agree to be accountable for all aspects of the manuscript.

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Correspondence to Stéphanie Clavé.

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Ethics approval and consent to participate

The study was approved by the ethics committee of the French Society of Paediatrics (CER_SFP_2018_079-3). Informed consent was obtained from the parent(s)/legal representative(s) of all individual participants included in the study, and, where appropriate, the patient provided written assent.

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The authors declare no competing interests.

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Communicated by Gregorio Paolo Milani

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Clavé, S., Sordet, M., Tsimaratos, M. et al. Association of kidney biopsy findings with short- and medium-term outcomes in children with moderate-to-severe IgA vasculitis nephritis. Eur J Pediatr 180, 3209–3218 (2021). https://doi.org/10.1007/s00431-021-04065-4

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