Diagnosis and Treatment of IgA Nephropathy and IgA Vasculitis Nephritis in Children

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Nephrology & Urology".

Deadline for manuscript submissions: closed (15 June 2023) | Viewed by 7552

Special Issue Editors


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Guest Editor
Azienda Ospedaliera - Universitaria Città della Salute e della Scienza di Torino, Turin, Italy
Interests: pediatric nephrology; children immune-mediated kidney disorders; IgA nephropathy and IgA vasculitis; pediatric kidney transplantation; congenital genetic based kidney diseases

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Guest Editor
Fondazione Ricerca Molinette, Nephrology, Dialysis and Transplantation, Regina Margherita Hospital, Turin, Italy
Interests: IgA nephropathy; IgA vasculitis; nephritis; pathogenesis; mesangial cells; therapy; pediatric nephrology

Special Issue Information

Dear colleagues,

IgA nephropathy (IgAN) is the most common glomerulonephritis at any age, starting from childhood. Pediatricians often deal with a mild disease, with recurrent macroscopic hematuria or persistent microscopic hematuria, rarely progressing to chronic kidney disease (CKD) stage 5 within their observation period.

IgA vasculitis is quite common in children, and the renal involvement—when present—is mostly self-limiting and benign, but in some cases is hampered by an explosive and aggressive onset or an even more dangerous smoldering progression.

After transition to adulthood the oft-neglected occurrence of proteinuria and hypertension may lead to the relentless loss of 50% of renal function in up to 40% of patients after 20 years in both diseases.

There is therefore a need to widen the perspective of pediatricians and pediatric nephrologists beyond the pediatric age to evaluate the risk of progression and adopt the best treatment to ensure the interruption of the progression of both diseases before the point of no return is reached.

The management of children requires the consideration of all the forecasted risk factors to choose the most appropriate treatment scheme and tailor the follow up.

In this Special Issue, an update on the available tools to estimate the risk factors for progression with the help of new biomarkers and a new look to old and well-known clinical and pathological risk factors will be provided to widen the point of view of pediatricians faced with the high responsibility of choosing the right treatment to provide a long and high-quality life expectancy.

We are soliciting new research papers on IgAN and IgAVN, including clinical, pathology, genetic biomarkers.

We also welcome the submission of brief state-of-the-art papers on therapy, genetics, biomarkers, and risk factor estimation tools.

Dr. Licia Peruzzi
Prof. Dr. Rosannna Coppo
Guest Editors

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Keywords

  • IgA nephropathy
  • IgA vasculitis nephritis
  • immunoglobulin A
  • pathogenesis
  • therapy
  • risk factors
  • progression

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Published Papers (2 papers)

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Research

9 pages, 691 KiB  
Article
Management of IgA Nephropathy in Pediatric Patients
by Sophie Schroda and Martin Pohl
Children 2022, 9(5), 653; https://doi.org/10.3390/children9050653 - 2 May 2022
Cited by 2 | Viewed by 3182
Abstract
The onset of IgA nephritis in childhood and adolescence often develops into chronic glomerulonephritis with declining renal function. Although these long-term consequences are known, there is still a lack of evidence-based treatment recommendations in this age group. We report data from 22 pediatric [...] Read more.
The onset of IgA nephritis in childhood and adolescence often develops into chronic glomerulonephritis with declining renal function. Although these long-term consequences are known, there is still a lack of evidence-based treatment recommendations in this age group. We report data from 22 pediatric patients who were biopsied to confirm the diagnosis of IgAN at our clinical center. 14 of them were treated with corticosteroids according to the recommendations for IgA nephritis vasculitis of the German Society of Pediatric Nephrology (GPN). Improvement was achieved in the majority of all cases, with a significant reduction in proteinuria five months after initiation of therapy. Our data suggest that treatment regimens for acute IgA nephritis and IgA vasculitis nephritis may be unified and are discussed in the context of current studies. Full article
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15 pages, 2218 KiB  
Article
Urinary Protein Array Analysis to Identify Key Inflammatory Markers in Children with IgA Vasculitis Nephritis
by Julien Marro, Andrew J. Chetwynd, Rachael D. Wright, Silothabo Dliso and Louise Oni
Children 2022, 9(5), 622; https://doi.org/10.3390/children9050622 - 27 Apr 2022
Cited by 4 | Viewed by 2896
Abstract
Chronic kidney disease is a recognised complication of immunoglobulin A vasculitis, (IgAV; formerly Henoch–Schonlein purpura—HSP). The pathophysiology of IgAV and why some patients develop significant renal involvement remains largely unknown. Identifying urinary inflammatory markers could direct targets for earlier intervention. The aim of [...] Read more.
Chronic kidney disease is a recognised complication of immunoglobulin A vasculitis, (IgAV; formerly Henoch–Schonlein purpura—HSP). The pathophysiology of IgAV and why some patients develop significant renal involvement remains largely unknown. Identifying urinary inflammatory markers could direct targets for earlier intervention. The aim of this cross-sectional exploratory study was to perform a large protein array analysis to identify urinary markers to provide insight into the mechanisms of kidney inflammation in children with established IgAV nephritis (IgAVN). Determination of the relative levels of 124 key proteins was performed using commercially available proteome profiler array kits. Twelve children were recruited: IgAVN, n = 4; IgAV without nephritis (IgAVwoN), n = 4; healthy controls (HCs), n = 4. The urinary concentrations of twenty proteins were significantly different in IgAVN compared to IgAVwoN. The largest fold changes were reported for B-cell activating factor (BAFF), Cripto-1, sex-hormone-binding globulin and angiotensinogen. The urinary levels of complement components C5/C5a and factor D were also significantly elevated in patients with IgAVN. A total of 69 urinary proteins significantly raised levels in comparisons made between IgAVN vs. HCs and nine proteins in IgAVwoN vs. HCs, respectively. This study identified key urinary proteins potentially involved in IgAVN providing new insight into the pathophysiology. Further longitudinal studies with larger cohorts are needed to quantitatively analyse these biomarkers. Full article
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