ABOUT THE DISEASE
Rapidly progressive glomerulonephritis (RPGN) is a disease of the kidney characterized clinically by a rapid decrease in the glomerular filtration rate (GFR) of at least 50% over a short period, from a few days to 3 months. (RPGN) is usually accompanied by microscopic glomerular crescent formation with progression to renal failure within weeks to months.
CAUSES
It occurs predominantly in patients 20 to 50 yr age and is classified pathologically into 3 categories, as follows: (1) anti-GBM antibody disease (approximately 3% of cases), (2) immune complex disease (45% of cases), and (3) pauci-immune disease (50% of cases).
Each of the above has varying degree of presence of ANCA - antineutrophil cytoplasmic antibodies (ANCAs).
The causes for each one of these vary:
- Anti-GBM antibody disease
Anti-GBM GN
Goodpasture syndrome
- Immune complex disease
Poststreptococcal GN
Infective endocarditis
Viral hepatitis B infection
Lupus nephritis
Henoch-Schonlein purpura
IgA nephropathy
- Pauci-immune disease
Churg-Strauss syndrome
Pulmonary necrotizing granulomas
Polyarteritis nodosa
SYMPTOMS
These include weakness, fatigue, fever, nausea, vomiting, anorexia, arthralgia, abdominal pain, nephrotic syndrome, oliguria, renal failure.
DIAGNOSIS
Diagnosis is suggested by acute kidney injury in patients with hematuria and dysmorphic RBCs or RBC casts. Testing includes serum creatinine, urinalysis, CBC, serologic tests, and renal biopsy.
TREATMENT METHODS
Options include corticosteroids and cyclophosphamide. Plasma exchange is recommended for anti-GBM antibody disease.
Renal transplant can be considered after disease activity is controlled.
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