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Rapidly progressive (crescentic) glomerulonephritis

  • 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, incidence, and risk factors

    The glomeruli are small structures inside the kidneys. They are the area where 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 show up as an acute nephritic syndrome or unexplained renal (kidney) failure. It often worsens rapidly to renal failure and end-stage renal disease.

    The disorder is more common in certain geographic areas. Mini-epidemics of this disorder have also occurred. It is most common in people aged 40-60, 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 developing 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.