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  • Review Article
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Henoch–Schönlein purpura nephritis in children

Nature Reviews Nephrology volume 10, pages 563–573 (2014)Cite this article

Subjects

Key Points

  • Henoch–Schönlein purpura (HSP)—the most common vasculitis in children—is complicated by nephritis in about 30% of patients

  • Long-term prognosis of HSP nephritis depends mainly on the development of chronic kidney disease (CKD); often CKD risk cannot be predicted from the initial clinical and histological presentation

  • CKD can be observed at long-term follow-up even after apparent complete recovery from HSP nephritis

  • HSP nephritis and IgA nephropathy both result from glomerular deposition of aberrantly glycosylated IgA1 but have different histological features and clinical courses

  • Typically IgA nephropathy presents as slowly progressive mesangial lesions, whereas HSP nephritis presents as acute episodes characterized by inflammatory glomerular lesions that require prompt resolution to avoid chronic progression

  • Use of guidelines based on evidence obtained in adults with IgA nephropathy to select treatment for children with HSP nephritis risks delaying the provision of adequate therapies

Abstract

Henoch–Schönlein purpura (HSP) is the most common vasculitis in children, in whom prognosis is mostly dependent upon the severity of renal involvement. Nephritis is observed in about 30% of children with HSP. Renal damage eventually leads to chronic kidney disease in up to 20% of children with HSP nephritis in tertiary care centres, but in less than 5% of unselected patients with HSP, by 20 years after diagnosis. HSP nephritis and IgA nephropathy are related diseases resulting from glomerular deposition of aberrantly glycosylated IgA1. Although both nephritides present with similar histological findings and IgA abnormalities, they display pathophysiological differences with important therapeutic implications. HSP nephritis is mainly characterized by acute episodes of glomerular inflammation with endocapillary and mesangial proliferation, fibrin deposits and epithelial crescents that can heal spontaneously or lead to chronic lesions. By contrast, IgA nephropathy normally presents with slowly progressive mesangial lesions resulting from continuous low-grade deposition of macromolecular IgA1. This Review highlights the variable evolution of similar clinical and histological presentations among paediatric patients with HSP nephritis, which constitutes a challenge for their management, and discusses the treatment of these patients in light of current guidelines based on clinical evidence from adults with IgA nephropathy.

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Figure 1: Pathogenesis of Henoch–Schönlein purpura nephritis.
Figure 2: Human IgA1 O-glycosylation sites and galactosylation patterns.

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Authors and Affiliations

  1. Emma Children's Hospital and Academic Medical Centre, University of Amsterdam, Meibergdreef 9, Amsterdam, 1105 AZ, Netherlands

    Jean-Claude Davin

  2. Nephrology, Dialysis and Transplantation Unit, Città della Salute e della Scienza di Torino, Regina Margherita University Hospital, Piazza Polonia 94, Turin, 1016, Italy

    Rosanna Coppo

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  1. Jean-Claude Davin
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The authors contributed equally to researching the data for the article, discussions of its content, writing the article and review and/or editing of the manuscript before submission.

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Davin, JC., Coppo, R. Henoch–Schönlein purpura nephritis in children. Nat Rev Nephrol 10, 563–573 (2014). https://doi.org/10.1038/nrneph.2014.126

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