Rapidly
Progressive (Crescentic) Glomerulonephritis
(RPGN)
Epidemiology:
Common sites:
Gross features:
- kidneys enlarged
and pale
- petichial hemorrhages on cortical surfaces
Histologic features:
- crescents in most glomeruli
- proliferation of
parietal epithelial cells
- infiltration of monocytes and macrophages
- neutrophils and lymphocytes may be present
- eventually
obliterate Bowman space and compress the glomerular
tuft
- prominent fibrin
strands between the cellular layers of the crescents
- focal glomerular necrosis
- diffuse or focal
endothelial proliferation
- mesangial proliferation
Immunophenotype:
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Marker:
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Sensitivity:
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Specificity:
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- 3
groups based on immunologic findings:
- Type I (Anti-GBM
antibody): linear deposits of IgG and (in
many) C3 at the GBM
- idiopathic
- Goodpasture syndrome
- Type II (Immune
complex): granular deposits
- Idiopathic
- Post-infectious
- Systemic
lupus erythematosus
- Henoch-Schonlein purpura (IgA)
- Others
- Type III (Pauci-immune): little or no deposition
- ANCA-associated
- Idiopathic
- Wegener
granulomatosis
- Microscopic
polyarteritis nodosa
/ microscopic polyangiitis
Molecular features:
Other features:
- Syndrome, not a
specific etiology
- Clinically:
- Rapid and
progressive loss of renal function
- Severe oliguria
- Hematuria
- Red cell casts
- Proteinuria
- Hypertension
- Edema
References: