Strategies for Addressing C3 Glomerulopathy (C3G) Disease

Strategies for Addressing C3 Glomerulopathy (C3G) Disease

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Hey there, let's dive into the latest treatments and future therapies for C3 Glomerulopathy (C3G), a rare kidney condition that affects around 2-3 individuals in every million. C3G causes a buildup of protein deposits in the kidney's filtering tissues, gradually impairing kidney function and sometimes leading to kidney failure.

As of now, there's no cure for C3G. Treatment kicks off by supporting healthy kidney function and reining in the immune system. Doctors often recommend systemic treatments to curb immune activity. For C3G, new and forthcoming treatments focus on targeting proteins implicated in disease activity.

What Causes C3G?

C3G happens when parts of the body's immune system ramp up beyond what's necessary. Genes play a significant role, creating proteins that manage the body's complement system, part of the immune system. When genes alter, they cause C3G.

Under normal circumstances, some of these proteins stay dormant until they encounter potentially hazardous bacteria and viruses. When this occurs, they ignite a sequence of events, activating a specific complement protein called C3, which triggers inflammation and opsonization – the preparation of harmful cells for destruction.

However, in C3G, these proteins become active more frequently, leading to an excessive amount of C3 protein. Parts of C3 turn into deposits in the kidney, , eventually causing damage to the glomeruli. The glomeruli are blood vessels in the kidneys that filter waste and extra fluid out of the blood. As C3 buildup ensues, there's progressive damage to the glomeruli, hindering the kidneys' ability to filter toxins out of the blood.

Besides genetic changes, most people with C3G carry antibodies hindering the complement system's normal function. Some evidence suggests genetic links between family members with the condition, but experts believe the genetic changes in C3G are not strictly inherited.

Current treatments for C3G can't undo or stop the condition. Instead, the goal is to slow down kidney damage.

Treatment Guidelines

Clinical guidelines from the Kidney Disease: Improving Global Outcomes (KDIGO) organization advise supportive interventions to help slow and prevent kidney damage. As kidney function deteriorates, the guidelines recommend immunosuppressive therapies.

ACE Inhibitors and ARBs

ACE inhibitors and ARBs are medications that lower blood pressure and also help prevent proteinuria – when a protein called albumin leaks through the kidneys' filters and into the urine.

Mycophenolate mofetil (MMF) and Glucocorticoids

MMF and glucocorticoids are medications that suppress the immune system. KDIGO guidelines suggest a person with C3G should receive immune-suppressing medications once they have had declining kidney function for at least six months.

In addition, these medications are recommended if a person has other indicators of C3G progression, such as increasing levels of protein in the urine.

Complement Inhibitors

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 – the last step of the complement pathway that causes cell death, as part of the innate immune response.

The use of eculizumab has had mixed results.

Dietary Factors

Following a diet that reduces the burden on the kidneys can be beneficial for people with C3G. They may want a diet that:

• reduces sodium, potassium, and phosphorus
• maintains a balance between protein and healthy fat levels
• strikes a balance between fluid intake

Some folks with kidney conditions opt to collaborate with a dietitian to create a diet plan that supports the kidneys while ensuring adequate nutrition.

Emerging Treatments

Emerging treatments for C3G target different aspects of the complement system. They interrupt the series of events that lead to the activation or breakdown of C3 or other proteins, with the objective of halting the damage C3G inflicts on the kidneys.

Several medicines in various stages of clinical trials include:

• pegcetacoplan (targets C3)
• ARO-C3 (targets C3)
• iptacopan (targets factor B)
• danicopan (targets factor D)
• avacopan (targets C5a)
• KP104 (targets C3 and C5)
• narsoplimab (targets MASP-2)

The Bottom Line

C3G is a rare kidney condition in which protein deposits hinder healthy kidney function. Currently, there's no cure for C3G.

Doctors aim to slow kidney damage in C3G by employing therapies that bolster kidney health and subdue the immune system. These advanced treatments in development focus on disrupting C3G activity as it impacts the complement system.

1. C3 Glomerulopathy (C3G) is an uncommon kidney disease that affects around 2-3 individuals per million, characterized by the buildup of excessive protein deposits in the kidney's filtering tissues.
2. C3G is a result of an overactive immune system, where genes play a significant role in creating proteins that manage the body's complement system, part of the immune system.
3. In C3G, these proteins become active more frequently, leading to an excessive amount of C3 protein which turns into deposits in the kidney, eventually causing damage to the glomeruli.
4. Current treatments for C3G can't undo or stop the condition, but aim to slow down kidney damage through supportive interventions.
5. Clinical guidelines from the Kidney Disease: Improving Global Outcomes (KDIGO) organization advise immunosuppressive therapies to slow and prevent kidney damage as kidney function deteriorates.
6. ACE inhibitors and ARBs are medications that lower blood pressure and help prevent proteinuria, and are recommended for people with C3G.
7. Mycophenolate mofetil (MMF) and glucocorticoids are immune-suppressing medications suggested for a person with C3G, once they have had declining kidney function for at least six months or have other indicators of C3G progression.
8. Complement inhibitors are a treatment option for C3G to slow down kidney damage, suggested when immunosuppressant medications are ineffective.
9. Following a diet that reduces the burden on the kidneys can be beneficial for people with C3G, which may include a diet that reduces sodium, potassium, and phosphorus, maintains a balance between protein and healthy fat levels, and balances fluid intake.
10. Emerging treatments for C3G target different aspects of the complement system, such as pegcetacoplan, ARO-C3, iptacopan, danicopan, avacopan, KP104, and narsoplimab, which aim to halt the damage C3G inflicts on the kidneys.
11. In health-and-wellness discussions, C3G is associated with various chronic diseases, autoimmune disorders, neurological disorders, skin conditions, respiratory conditions, digestive health, eye-health, hearing, cardiovascular-health, and malignancies (cancer), emphasizing the interconnectedness of these medical conditions. CBD oil, although not directly related to C3G, is often discussed within the context of health and wellness, offering potential benefits for managing certain symptoms and conditions.

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