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C3 Glomerulopathy (C3G)

What is C3 glomerulopathy (C3G)?

C3 glomerulopathy is a group of related conditions that cause the kidneys to malfunction. The major features of C3 glomerulopathy include high levels of protein in the urine (proteinuria), blood in the urine (hematuria), reduced amounts of urine, low levels of protein in the blood, and swelling in many areas of the body. Affected individuals may have particularly low levels of a protein called complement component 3 (or C3) in the blood. Researchers have identified two major forms of C3 glomerulopathy: dense deposit disease and C3 glomerulonephritis. Although the two disorders cause similar kidney problems, the features of dense deposit disease tend to appear earlier than those of C3 glomerulonephritis, usually in adolescence. However, the signs and symptoms of either disease may not begin until adulthood.

What are the symptoms C3 glomerulopathy (C3G)?

The following are characteristic symptoms of C3G:

• Blood in the urine (hematuria)
• Dark foamy urine, which signifies the presence of protein or proteinuria
• Cloudiness of the urine, reflecting the presence of white blood cells
• Swelling or ‘edema’, initially of the legs although any part of the body can be affected
• High blood pressure
• Decreased urine output
• Decreased alertness

What causes C3 glomerulopathy (C3G)?

The immediate cause of the symptoms of C3G is the change in the filtering mechanism of the kidney. The damaged glomeruli (the filters) permit protein and red and white blood cells to pass into the urine-containing space. The specific cause of C3G is lack of regulation of the complement system. The causes of complement dysregulation can be divided into genetic and acquired factors.

How is C3 glomerulopathy (C3G) diagnosed?

C3G can only be diagnosed by a kidney biopsy. The kidney deposits stain for the complement protein C3 and when examined under an electron microscope, dense deposits are present.

What are the treatments for C3 glomerulopathy (C3G)?

There is currently no specific therapy for C3G, however a number of non-specific treatments are appropriate. These treatments slow progression of chronic glomerular diseases through aggressive blood pressure control and reduction of proteinuria.

Where can I find out more about C3 glomerulopathy (C3G)?

• NIH
• National Organizations for Rare Disorders (NORD)
• Rare Renal

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