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Rapidly Progressive Glomerulonephritis

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Pediatric Kidney Disease

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Abstract

Rapidly progressive glomerulonephritis (RPGN) is characterized by clinical features of glomerulonephritis (GN) and rapid loss of renal function. Histology shows crescentic extracapillary proliferation in Bowman space affecting the majority of glomeruli. A rapidly progressive illness may occur in any form of GN, including postinfectious GN, renal vasculitis, IgA nephropathy and membranoproliferative GN. The combination of renal histology and serological investigations enables diagnosis of the underlying etiology. Guidelines for therapy of RPGN have chiefly been adapted from studies in adults. Apart from renal replacement therapy and supportive care, specific therapy comprises a combination of high dose intravenous followed by tapering oral corticosteroids and other immunosuppressive medications. Patients with underlying systemic vasculitis and anti-glomerular basement membrane crescentic GN benefit from prompt plasma exchanges. Outcomes in patients with RPGN depend on the underlying cause and the promptness of institution of therapy.

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Authors and Affiliations

  1. Department of Pediatrics, University of Washington, Seattle, WA, USA

    Shina Menon

  2. Division of Nephrology, Seattle Children’s Hospital, Seattle, WA, USA

    Shina Menon

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

    Arvind Bagga

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  1. Shina Menon
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  2. Arvind Bagga
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Correspondence to Shina Menon .

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Editors and Affiliations

  1. Division of Pediatric Nephrology, Heidelberg University, Heidelberg, Germany

    Franz Schaefer

  2. Emory School of Medicine, Emory University, Atlanta, GA, USA

    Larry A. Greenbaum

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Menon, S., Bagga, A. (2023). Rapidly Progressive Glomerulonephritis. In: Schaefer, F., Greenbaum, L.A. (eds) Pediatric Kidney Disease. Springer, Cham. https://doi.org/10.1007/978-3-031-11665-0_20

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