This is true for IgA nephropathy. It is not a disease that is routinely tested for in many countries (such as the United States), and although it seems to run in families, it can occur in anyone and not be associated with any other disease.
What is it?
IgA nephropathy is a form of glomerulonephritis. Damage is caused in the kidney by the abnormal buildup of a protein (IgA). Research suggests that this is due to an autoimmune disorder (involving the body's immune system). What starts the disease in motion is not yet known.
Who gets it?
In the United States, Canada, and the United Kingdom, IgA nephropathy accounts for 2-10% of glomerular kidney diseases, but in Japan, it accounts for 18-40%. It often begins in older children or young adults in their 20s and 30s. The disease occurs more often in males, and is rare in blacks. It seems to run in families, which suggests that genetics play a part in who develops it.
How is it diagnosed?
The most common symptom of IgA nephropathy is blood in the urine, which causes the urine to look tea-colored. To confirm the diagnosis, a small piece of kidney tissue must be removed (biopsy) and examined. There is some concern that IgA nephropathy may actually be less rare than thought, since some people may have it but without the biopsy the disease wouldn't be detected.
How is it treated?
One of the most serious consequences of IgA nephropathy is that the kidney becomes so damaged that it stops working, called end-stage kidney failure or end-stage renal disease (ESRD). This will happen in about 30-40% of people with the disease. There is a lack of agreement among researchers about how to best prevent ESRD from happening in IgA nephropathy. Some of the current treatments are:
- Control of high blood pressure with diet and medication
- Steroids such as prednisone
- Drugs to suppress the immune system, such as Imuran, Cytoxan, or CellCept
- Fish oil dietary supplements
What is the outlook?
As many as 50% of people with IgA nephropathy don't develop serious disease. Studies are trying to discover how to best prevent the disease from becoming kidney failure, or even how to reverse the disease's effects. Future research could look for how the disease occurs, and how to easily test for its presence so that early treatment could be started.
Information for this article was taken from:
- IgA Nephropathy Foundation. What is IgA Nephropathy?
- National Kidney Foundation. IgA Nephropathy.
- Julian, B. A. (2000). Treatment of IgA nephropathy. Semin Nephrol, vol. 20, no. 3, pp 277-285.
- Scolari, F. (1999). Familial IgA nephropathy. Journal of Nephrology, vol. 12, no. 4, pp 213-219.