Review Article
Rapidly Progressive Glomerulonephritis

https://doi.org/10.1053/j.akdh.2024.08.006Get rights and content
Rapidly progressive glomerulonephritis (RPGN) is a syndrome characterized by a swift decline in kidney function, often over a few months, accompanied by features of nephritic syndrome. It can result in decreased urine output and commonly involves the presence of extensive crescents in kidney biopsies. RPGN is classified into 3 main types based on immune deposit distribution and visualization through immunofluorescence and electron microscopy: antiglomerular basement membrane disease, immune complex glomerulonephritis, and pauci-immune glomerulonephritis. Early diagnosis and prompt treatment are critical to prevent progression to ESRD. Standard treatment options for RPGN include glucocorticoids, cyclophosphamide, or rituximab, with plasma exchange especially important for antiglomerular basement membrane disease and select cases of ANCA-associated vasculitis. Clinical trials for glomerular diseases have primarily excluded patients with RPGN or dialysis dependence. Establishment of clinical registries is required for the optimization of therapeutic protocols for the treatment of RPGN.

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CASE 1

An 82-year-old man with a history of hypertension and coronary artery disease presents with significant fatigue, lethargy, and elevated blood pressure of 150/90. Lab tests show a drastic increase in creatinine from 1.2 to 8.0 mg/dL, significant hematuria (too numerous to count/HPF), proteinuria (0.8 gms/gm of creatinine), hemoglobin of 12 g/dL, and positive antiglomerular basement membrane antibodies. Chest X-ray reveals mild pulmonary congestion but no lung infiltrates. He starts hemodialysis

SUMMARY

RPGN is a syndrome marked by rapid kidney function decline within days to a few months and nephritic syndrome features, often leading to oliguria and characterized by extensive crescents in kidney biopsies. It is categorized into anti-GBM disease, immune complex GN, and pauci-immune GN based on the presence and distribution of immune deposits by immunofluorescence and electron microscopy. Early diagnosis and treatment are crucial to avoid ESRD. Standard RPGN treatment includes glucocorticoids

Acknowledgments

We thank Dr Behzad Najafian, a Professor of Laboratory Medicine and Pathology at the University of Washington in Seattle, for generously providing us with kidney biopsy photographs.

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    Financial Disclosure: The authors declare that they have no relevant financial interests.
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