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Classification of Rapidly Progressive Glomerulonephritis Based on Immunofluorescence Microscopy

Type

Approximate Percentage of RPGN Cases*

Causes

Anti-GBM antibody–mediated

20%

Anti-GBM GN (without lung hemorrhage†)

Goodpasture syndrome (with lung hemorrhage)

Immune complex-mediated

50%

Postinfectious causes:

Systemic rheumatic diseases:

Other glomerulopathies:

Pauci-immune

30%

Granulomatosis with polyangiitis

Renal-limited vasculitis (eg, idiopathic crescentic GN)

Microscopic polyangiitis

Double-antibody positive

Rare

Same as for anti-GBM antibody-mediated and pauci-immune types

* Data from Satoskar AA, Parikh SV, Nadasdy T. Epidemiology, pathogenesis, treatment and outcomes of infection-associated glomerulonephritis. Nat Rev Nephrol 2020;16(1):32-50. doi:10.1038/s41581-019-0178-8

† When the lung is also affected, anti-GBM glomerulonephritis is called Goodpasture syndrome.

GBM = glomerular basement membrane; GN = glomerulonephritis; RPGN = rapidly progressive glomerulonephritis; IgA = immunoglobulin A.