The topic C3 Glomerulonephritis (C3GN) you are seeking is a synonym, or alternative name, or is closely related to the medical condition C3 Glomerulopathy.

Quick Summary:

• C3 Glomerulopathy refers to a group of rare kidney-related disorders that has 2 major subtypes:
  • C3 Glomerulonephritis (C3GN)
  • C3 Glomerulopathy-Dense Deposit Disease (C3G-DDD)
• Both are differentiated on the basis of electron microscopy. The mean age of onset for DDD is earlier than that of C3GN, despite the fact that both can occur at any age
• Most cases of C3 Glomerulopathy have no known cause and the risk factors for such cases are currently unknown. A major risk factor for the inherited version of C3 Glomerulopathy is a family history of the condition
• In addition, a general family history of autoimmune disorders predisposes an individual to C3G. Nonetheless, an exact correlation between the two conditions is still not clearly understood
• The causes for most of the cases of C3 Glomerulopathy are still unknown. Mutations in the C3, CFH, and CFHR5 genes of the C3 complement system are known to cause this kidney disorder in certain cases. The complement system is a component of a body’s immune response involved in responding to invading entities such as bacteria and viruses
• The mutations in the aforementioned genes are known to deregulate the C3 system, leading it to become overactive. This leads to depositions of C3 protein due to damage to the cluster of blood vessels within the kidneys known as the glomeruli, which filter waste from the bloodstream
• The damage to the glomeruli results in the major symptoms of C3 Glomerulopathy including blood and protein in urine, reduced urine output, decrease in general alertness, and high blood pressure. Those with DDD also may have acquired lipodystrophy (lack of fat deposits in the body’s upper region) and deposits of a yellowish substance in the retina
• Some of the complications of C3 Glomerulopathy include progressive kidney damage leading to kidney failure, end-stage renal disease, and vision abnormalities
• A diagnosis of C3G may require a physical examination, an assessment of symptoms, an evaluation of family history, urine analysis, kidney biopsy, testing for blood C3 levels, and genetic testing
• There is currently no cure available for C3 Glomerulopathy; however, steps can be taken to slow the progression of the disorder. Treatment options can include medication to control the high blood pressure, use of steroids and other immunosuppressants, as well as monoclonal antibody-based therapy. When kidney failure ensues, dialysis and kidney transplantation become available options for treatment
• Thus far, no preventive methods or guidelines have been described for this disease. If there is a known family history of either C3 Glomerulopathy or autoimmune disorders, then it may be necessary to proceed with genetic testing of the fetal cells. This may allow the expecting parents to understand the disorder and its risks better
• Since C3 Glomerulopathy is a progressive disease, the prognosis is guarded. The 10-year life expectancy after diagnosis is approximately 65%. Even with kidney transplantation, there remains a 70% probability of the disease recurring in the transplanted kidney

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