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Review
. 2023 Mar 7:14:89-98.
doi: 10.2147/PHMT.S379862. eCollection 2023.

Diagnostic and Management Strategies of IgA Vasculitis Nephritis/Henoch-Schönlein Purpura Nephritis in Pediatric Patients: Current Perspectives

Mario Sestan  1 , Marija Jelusic  1
Affiliations
Review

Diagnostic and Management Strategies of IgA Vasculitis Nephritis/Henoch-Schönlein Purpura Nephritis in Pediatric Patients: Current Perspectives

Mario Sestan et al. Pediatric Health Med Ther. .

Abstract

IgA vasculitis (IgAV) or Henoch-Schönlein purpura (HSP) is the most common vasculitis in children, and nephritis (IgAVN or HSPN) is the most important and only chronic manifestation of the disease. Despite this, there are no diagnostic criteria and we rely on the European League Against Rheumatism/Paediatric Rheumatology International Trials Organization/Paediatric Rheumatology European Society-endorsed Ankara 2008 classification criteria in our daily practice. Basic investigations that should be done in every patient with IgAVN include blood pressure measurement, estimated glomerular filtration rate and urinalysis. Kidney biopsy is still the gold standard for the diagnosis of IgAVN since noninvasive confirmation of nephritis is still pending. According to the Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) recommendations, the first-line treatment for with mild forms of IgAVN is oral glucocorticoids, for patients with moderate IgAVN parenterally administrated glucocorticoids in pulsed doses, while initial treatment for patients with the most severe forms of IgAVN include pulsed doses of glucocorticoids in combination with intravenous cyclophosphamide pulses. New therapeutic options are currently being tested, aiming to reduce the production of galactose-deficient IgA1 and autoantibodies or suppress the alternative or lectin complement pathway and blocking mesangial cell activation.

Keywords: IgA vasculitis nephritis; clinical manifestations; hematuria; proteinuria; treatment.

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Conflict of interest statement

The authors report no conflicts of interest in this work.

Figures

Figure 1
Figure 1
Diagnostic approach to IgAV and IgAVN. Data from Ozen et al. Adapted from Jelusic M, Sestan M, Giani T, Cimaz R. New insights and challenges associated with IgA vasculitis and IgA vasculitis with nephritis-is it time to change the paradigm of the most common systemic vasculitis in childhood? Front Pediatr. 2022;10:853724. Creative commons.
Figure 2
Figure 2
The SHARE recommendations for IgAVN treatment. Data from Ozen et al. Jelusic M, Sestan M, Giani T, Cimaz R. New insights and challenges associated with IgA vasculitis and IgA vasculitis with nephritis-is it time to change the paradigm of the most common systemic vasculitis in childhood? Front Pediatr. 2022;10:853724. Creative Commons.
See this image and copyright information in PMC

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