Definition
Rapidly progressive glomerulonephritis is a form of
kidney disease that causes damage to the internal structures of the kidneys and rapid loss of function, with crescent-shaped abnormalities showing on a
biopsy of the kidney.
Alternative Names
Necrotizing glomerulonephritis; Glomerulonephritis - crescentic; Crescentic glomerulonephritis
Causes
The glomeruli are the portions of the internal kidney structures where the blood flows through very small capillaries and is filtered through membranes to form urine.
Rapidly progressive glomerulonephritis includes any type of
glomerulonephritis (inflammation of the glomerulus) in which progressive loss of kidney function occurs over weeks to months. Most pathologists define crescentic glomerulonephritis when 50% or more glomeruli reveal crescents on kidney biopsy. It may manifest itself as an
acute nephritic syndrome or unexplained
renal failure. It often progresses rapidly to renal failure and
end-stage renal disease.
The disorder occurs with varying incidences in different geographic regions. Mini-epidemics of this disorder have also been described. It is most common in people 40 to 60 years old, and slightly more common in men but, depending on the cause, occurs in both sexes and at any age. It is unusual in preschool children, and slightly more common in later childhood.
Many conditions are known to cause or increase the risk for development of this syndrome. These include vascular (blood vessel) diseases such as
vasculitis or polyarteritis,
abscess of any internal organ,
collagen vascular disease such as
lupus nephritis and
Henoch-Schonlein purpura,
Goodpasture's syndrome,
IgA nephropathy, membranoproliferative GN,
anti-glomerular basement membrane antibody disease, a history of malignant tumors or blood or lymphatic system disorders, and exposure to hydrocarbon solvents. The symptoms are similar regardless of the cause.
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