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rapidly progressive glomerulonephritis

Alternative Names
RPGN, crescentic glomerulonephritis

Definition
Rapidly progressive glomerulonephritis (RPGN) is a rapid deterioration in kidney function over a short time. This condition usually occurs when there is an acute inflammation of the kidneys.

What is going on in the body?
RPGN is linked to an immune reaction against the glomerulus, the filtering unit of the kidney. Antibodies that form against an infection can become trapped in the glomeruli. The resulting inflammation causes protein and blood to be excreted in the urine. The ability of the kidneys to get rid of toxic wastes is impaired.

What are the signs and symptoms of the disease?
Symptoms of RPGN include: Uraemia may cause: What are the causes and risks of the disease?
The causes of RPGN include: What can be done to prevent the disease?
Depending on the cause, some cases of RPGN may be prevented. Early treatment of other conditions, such as systemic lupus erythematosus or hepatitis, may help prevent RPGN from these causes. In other cases, RPGN cannot be prevented.

How is the disease diagnosed?
Diagnosis of RPGN starts with a medical history and a complete physical examination. Urine tests will also be done. High levels of protein and red blood cells in the urine indicate RPGN. The diagnosis is confirmed by a kidney biopsy. The biopsy is very useful, because it can show the type and severity of disease. Other laboratory tests and blood cultures may also be done.

What are the long-term effects of the disease?
Long-term effects of RPGN vary, depending on the underlying cause. For example, hepatitis may cause long-term effects in the liver and kidneys. An infection that is treated promptly may cure the cause of the RPGN, and there may be no long-term effects. The longer the condition goes untreated, the more devastating it can be. If it is not treated, the kidneys and renal system can be permanently damaged. End-stage renal disease, the permanent and complete loss of kidney function, can occur within days or weeks of the start of RPGN.

What are the risks to others?
RPGN is not contagious by itself, but the underlying cause may be contagious. For example, if cause of the RPGN is hepatitis, the hepatitis can be spread to others.

What are the treatments for the disease?
Treatment of RPGN depends on the underlying cause. Corticosteroids or other medications that suppress the immune system may be used. Plasmapheresis, or blood filtering, may be done. The person may need bed rest and restricted fluid and salt intake, to give the kidneys a chance to heal. Treatment for the underlying cause, such as antibiotics for a strep infection, may also be needed.

What are the side effects of the treatments?
Corticosteroids can cause fluid retention, swelling, weight gain, and increased risk of infection. Taking corticosteroids for a long time can cause osteoporosis, or bone thinning. Some of the more aggressive medication treatments may increase the risk of infections and cancer.

What happens after treatment for the disease?
Early treatment of RPGN is essential to avoid kidney damage. Severe RPGN can result in end-stage renal disease.

How is the disease monitored?
Close follow-up with the doctor is needed. Regular blood tests can help monitor for recurrences of RPGN. Kidney function should also be monitored regularly. Any new or worsening symptoms should be reported to the doctor.

Author: Crystal R. Martin, MD
Reviewer: HealthAnswers Australia Medical Review Panel
Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia
Last Updated: 1/10/2001
Contributors
Potential conflict of interest information for reviewers available on request


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