Crescentic glomerulonephritis
| Rapidly progressive glomerulonephritis | |
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| Histopathological image of crescentic glomerulonephritis in a patient with MPO-ANCA positive rapid progressive glomerulonephritis. Hematoxylin & eosin stain. | |
| Specialty | Nephrology |
| Types | Type I, II and III |
| Diagnostic method | Serum analysis |
| Treatment | Corticosteroids and Cyclophosphamide |
| Classification |
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| External resources |
Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that is characterized by a rapid loss of renal function, (usually a 50% decline in the glomerular filtration rate (GFR) within 3 months) with glomerular crescent formation seen in at least 50% or 75% of glomeruli seen on kidney biopsies. If left untreated, it rapidly progresses into acute renal failure and death within months. In 50% of cases, RPGN is associated with an underlying disease such as Goodpasture syndrome, systemic lupus erythematosus or granulomatosis with polyangiitis; the remaining cases are idiopathic. Regardless of the underlying cause, RPGN involves severe injury to the kidneys' glomeruli, with many of the glomeruli containing characteristic glomerular crescents (crescent-shaped scars).
Most types of RPGN are characterized by severe and rapid loss of kidney function featuring severe hematuria (blood in the urine), red blood cell casts in the urine, and proteinuria (protein in the urine), sometimes exceeding 3 g protein/24 h, a range associated with nephrotic syndrome. Some patients also experience hypertension (high blood pressure) and edema. Severe disease is characterized by pronounced oliguria or anuria, which portends a poor prognosis.
In the pathophysiology of rapidly progressive glomerulonephritis the antineutrophil cytoplasmic antibodies (ANCA) interact with antigens in cytoplasm of neutrophils. It is thought that ANCA causes an early degranulation giving way to release of lytic enzymes at site of injury. ANCA are linked to the pathogenesis of glomerulonephritis, antineutrophil cytoplasmic antibodies specificity is determined via (ELISA), with pANCA(antibody) directed against MPO
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