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Rapidly progressive glomerulonephritis

Last updated: April 28, 2021

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Rapidly progressive glomerulonephritis (RPGN) is an inflammatory disease of the kidneys characterized by rapid destruction of the renal glomeruli that often leads to end-stage renal disease. There are three different pathophysiological mechanisms that can result in RPGN: anti-glomerular basement membrane antibody disease (Goodpasture syndrome), immune complex glomerulonephritis (e.g., lupus nephritis), and glomerulonephritis associated with vasculitis (e.g., granulomatosis with polyangiitis). Patients may present with mild symptoms such as flank pain, edema, and decreased urine output. Laboratory tests show a sudden rise in serum BUN and creatinine as well as RBC casts and dysmorphic red blood cells on urinalysis. If the patient presents with hemoptysis, pulmonary-renal syndrome should be suspected. RPGN requires rapid diagnosis and immediate initiation of immunosuppressive therapy to prevent irreversible kidney damage.

References:[1]

References:[2]

References:[1][3]

Characteristics of RPGN
Anti-GBM disease Immune complex-mediated glomerulonephritis Glomerulonephritis associated with vasculitis (pauci-immune GN)
Laboratory findings
Serology
Biopsy findings

If serum creatinine rises rapidly due to renal damage, always suspect RPGN and initiate testing immediately. If urinalysis shows nephritic sediment, a renal biopsy is vital for quick diagnosis and initiation of appropriate treatment.

Differential diagnosis of pulmonary-renal syndromes
Goodpasture syndrome Granulomatosis with polyangiitis Churg-Strauss syndrome Microscopic polyangiitis Systemic lupus erythematosus
Pathophysiology
Clinical findings
  • Mild respiratory symptoms
Nephritis

Laboratory findings

  • Anti-GBM antibodies


Elevated renal function parameters and hemoptysis are a red flag for RPGN.

References:[1]

The differential diagnoses listed here are not exhaustive.

References:[1]

  1. Goldman L, Schafer AI. Goldman-Cecil Medicine, 25th Edition. Elsevier ; 2016
  2. Kumar V, Abbas AK, Aster JC. Robbins & Cotran Pathologic Basis of Disease. Elsevier Saunders ; 2015
  3. Longo D, Fauci A, Kasper D, Hauser S, Jameson J, Loscalzo J. Harrisons's Principles of Internal Medicine, 18th Edition, 2011. McGraw-Hill Medical ; 2011