Rapidly progressive (crescentic) glomerulonephritis

Alternative names:

crescentic glomerulonephritis; glomerulonephritis - crescentic; necrotizing glomerulonephritis

Definition:

A form of kidney disease causing damage to the internal structures (particularly the glomeruli) and rapid loss of kidney function, with crescents ("new-moon shaped" abnormalities) showing on a biopsy of the kidney.

Causes, incidence, and risk factors:

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 days to weeks rather than months to years, and in which a kidney biopsy shows crescents in at least 75% of the glomeruli. It may manifest itself as an acute nephritic syndrome or unexplained renal failure. It progresses rapidly to renal failure and end-stage renal disease.

The disorder occurs in about 1 out of 10,000 people. 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.