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Henoch Schönlein Purpura Nephropathy

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Pediatric Nephrology

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Abstract

IgA vasculitis, formerly known as Henoch-Schönlein purpura (HSP), is a clinical disorder defined as palpable purpura with one or more of arthritis/arthralgia, abdominal pain, renal involvement, or predominant IgA deposition. IgA vasculitis largely affects the pediatric population and generally has a favorable prognosis, with IgA vasculitis with nephritis being a feared outcome that contributes to poor prognosis. Renal involvement has a large range of symptoms, from microscopic hematuria to mixed nephritic-nephrotic syndrome with diminished renal function. The pathogenesis of IgA vasculitis with nephritis is similar to IgA nephropathy, suggesting a role of galactose-deficient IgA1 (GdIgA1) acting as an autoantigen, resulting in immune complex formation and deposition within the kidneys. Histology is indistinguishable between IgA vasculitis with nephritis and IgA nephropathy, with crescents and mesangial hypercellularity being hallmark features of IgA vasculitis with nephritis. Treatment recommendations are aimed at treatment of proteinuria when present, as well as attempting to reduce nephritis to prevent long-term damage by treatment with corticosteroids and immunosuppressive medications. Studies are currently ongoing to review clinical and histological factors that may suggest poor prognosis, with degree of proteinuria an important marker for outcome. Children with a history of IgA vasculitis with nephritis require long-term monitoring for adverse outcomes including hypertension, CKD, and ESKD.

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Authors and Affiliations

  1. Department of Child Health and Welfare (Pediatrics), Graduate School of Medicine, University of the Ryukyus, Nishihara-cho, Japan

    Koichi Nakanishi

  2. Pediatric Nephrology Department, Regina Margherita Children’s University Hospital, Health and Science City of Turin, Turin, Italy

    Licia Peruzzi

  3. Department of Pathology, Microbiology and Immunology, Division of Renal/Electron Microscopy, Vanderbilt University Medical Center, Nashville, TN, USA

    Agnes B. Fogo

  4. Division of Nephrology, University of Michigan, Ann Arbor, MI, USA

    Brooke Blazius & Debbie Gipson

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  1. Koichi Nakanishi
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  2. Licia Peruzzi
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  3. Agnes B. Fogo
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  4. Brooke Blazius
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  5. Debbie Gipson
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Correspondence to Debbie Gipson .

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Editors and Affiliations

  1. Division of Nephrology, Department of Pediatric Specialties, Bambino Gesù Children’s Hospital – IRCCS, Rome, Italy

    Francesco Emma

  2. Division of Nephrology and Hypertension, The Heart Institute, Cincinnati Children’s Hospital Medical Center, College of Medicine, Cincinnati, OH, USA

    Stuart L. Goldstein

  3. Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India

    Arvind Bagga

  4. Division of Pediatric Nephrology, UPMC Children’s Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

    Carlton M. Bates

  5. Department of Paediatric Nephrology, Great Ormond Street Hospital for Children, NHS Foundation Trust and University College London Institute of Child Health, London, UK

    Rukshana Shroff

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Nakanishi, K., Peruzzi, L., Fogo, A.B., Blazius, B., Gipson, D. (2022). Henoch Schönlein Purpura Nephropathy. In: Emma, F., Goldstein, S.L., Bagga, A., Bates, C.M., Shroff, R. (eds) Pediatric Nephrology. Springer, Cham. https://doi.org/10.1007/978-3-030-52719-8_96

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