Exploration of Remedial Strategies for C3 Glomerulopathy (C3G)
A Rare Disorder Affects Kidney Function: C3 Glomerulopathy (C3G)
C3 Glomerulopathy (C3G) is a seldom seen kidney condition that impacts approximately 2-3 individuals per million. This ailment leads to the accumulation of protein deposits in the kidney's filtering tissues, eventually impairing kidney function and potentially causing kidney failure.
As there is no known cure for C3G, treatment begins with strategies to maintain healthy kidney function. Medical professionals often recommend systemic treatments to suppress the immune system. New and emerging treatments for C3G target proteins tied to disease activity.
The causes of C3G stem from overactive parts of the body's immune system. Genes create proteins essential for managing the body's complement system, a part of the immune system. When genes mutate, this causes C3G. In the absence of C3G, some proteins remain inactive but become active when encountering harmful bacteria or viruses. In C3G, these proteins become overactive, leading to an excess of C3 protein. Parts of the C3 protein turn into deposits in the kidney, affecting the glomeruli. The glomeruli are blood vessels located in the kidneys, which filter waste and excess fluids from the blood. The buildup of C3 causes progressive damage to the glomeruli, hindering the kidneys' ability to filter toxins from the blood.
In addition to genetic changes, most individuals with C3G carry antibodies that impair the complement system's regular function. There is some evidence of genetic links between family members with the condition. However, experts believe that the genetic changes in C3G are not strictly inherited.
Current treatments for C3G cannot reverse or prevent the condition. Instead, the objective of treatment is to slow down kidney damage. Clinical guidelines from the Kidney Disease: Improving Global Outcomes (KDIGO) organization for managing C3G recommend supportive interventions to help slow and prevent kidney damage. As kidney function declines, the guidelines recommend immunosuppressive therapies.
Angiotensin converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) are medications that lower blood pressure and help prevent proteinuria, a condition where protein leaks through the kidneys' filters and into the urine.
Mycophenolate mofetil (MMF) and glucocorticoids are both medications that suppress the immune system. The KDIGO guidelines recommend a person with C3G receives immune-suppressing medications once they have had declining kidney function for at least six months.
Doctors consider complement inhibitors a treatment option for C3G to slow down kidney damage. This type of medication stops complement system activity. A doctor may suggest these medications to treat C3G if immunosuppressant medications are ineffective. Eculizumab and ravulizumab are monoclonal antibodies that block the activity of the complement system's terminal pathway.
Eating certain foods can help reduce the burden on the kidneys. A person with C3G might follow a diet that reduces sodium, potassium, and phosphorus, balances protein and healthy fat levels, and balances fluid intake. Some people with kidney conditions choose to work with a dietitian who can help create a diet plan that supports the kidneys while also ensuring adequate nutrition.
Research is underway for new treatments for C3G. Some medicines in various stages of clinical trials include pegcetacoplan, ARO-C3, iptacopan, danicopan, avacopan, KP104, and narsoplimab. These treatments target different parts of the complement system, with the aim of preventing the damage that C3G does to the kidneys.
The treatment landscape for C3G is evolving, with the introduction of new drugs and ongoing research aimed at developing more effective therapies for rare kidney diseases.
- C3 Glomerulopathy (C3G) is an uncommon kidney disease that belongs to the category of chronic diseases.
- The causes of C3G can be traced back to overactive parts of the immune system, which are controlled by genes that produce essential proteins for managing the body's complement system.
- In addition to genetic changes, most individuals with C3G carry antibodies that impair the regular function of the complement system.
- The accumulation of protein deposits in the kidney's filtering tissues, due to C3G, can lead to respiratory conditions and digestive health issues.
- ACE inhibitors and angiotensin receptor blockers (ARBs) are medications used to help prevent proteinuria, a condition caused by excess protein in the urine.
- Mycophenolate mofetil (MMF) and glucocorticoids are immune-suppressing medications used in the treatment of C3G once kidney function has started declining.
- Complement inhibitors, such as eculizumab and ravulizumab, are being considered as a treatment option for C3G as they can slow down kidney damage by stopping the activity of the complement system.
- Research is being conducted on new treatments for C3G, focusing on medicines like pegcetacoplan, ARO-C3, iptacopan, danicopan, avacopan, KP104, and narsoplimab, which aim to prevent the damage caused by C3G to the kidneys, potentially improving the health and wellness of those affected by this rare kidney disease, and even extending to other medical-conditions such as autoimmune-disorders, neurological-disorders, cardiovascular-health, skin-conditions, eye-health, and hearing impairments.
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