Methods for Managing C3 Glomerulopathy (C3G) Condition
Rare Kidney Condition, C3 Glomerulopathy (C3G), Treated to Support Kidney Function and Curb Immune Activity
Affecting approximately 2 to 3 individuals in every million, C3G is a rare kidney condition characterized by the buildup of protein deposits in the filtering tissues of the kidney. Over time, this accumulation impairs kidney function and can ultimately lead to kidney failure.
Current treatments for C3G aim to support healthy kidney function and suppress the immune system. Systemic treatments are recommended to achieve this, with emerging therapies targeting proteins that contribute to disease activity.
The causes of C3G are linked to genetic changes that affect certain genes producing proteins that manage the body's complement system, a part of the immune system. Changes in these genes cause an overactive immune response, leading to an excess of C3 protein and subsequent C3 buildup in the kidneys, which damages the glomeruli. These blood vessels, located within the kidneys, filter waste and excess fluid from the blood.
Other than genetic changes, people with C3G may carry antibodies that impair the regular function of the complement system. There are links between family members with the condition, although these genetic changes are not strictly inherited.
As there is currently no cure for C3G, treatments focus on slowing down kidney damage to prolong kidney function. Clinical guidelines from the Kidney Disease: Improving Global Outcomes (KDIGO) organization recommend supportive interventions like angiotensin converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) for slowing and preventing kidney damage.
When kidney function declines, immunosuppressive therapies like mycophenolate mofetil (MMF) and glucocorticoids are recommended. These medications are also suggested if a person shows other markers of C3G progression, such as increasing levels of protein in the urine.
Complement inhibitors are another treatment option for C3G, as they can slow down kidney damage by stopping complement system activity. They may be suggested for treating C3G if immunosuppressant medications are ineffective.
Diet can help reduce the burden on the kidneys by consuming a diet rich in nutrients that are easier on kidney function. Such diets may reduce sodium, potassium, and phosphorus while balancing protein and healthy fat levels. Furthermore, these diets can balance fluid intake and may require the assistance of a dietitian.
Research is ongoing for C3G therapies, and emerging treatments aim to interrupt C3G activity as it affects the complement system. Some medications currently in 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), and narsoplimab (targets MASP-2).
These latest advancements in C3G treatment offer hope for patients by focusing on emergency therapies that target proteins responsible for disease activity. Recent research has led to the approval of iptacopan, the first and only medication approved by the U.S. FDA for treating C3G. pegcetacoplan, another promising treatment, is awaiting an FDA decision, while ongoing and future clinical trials highlight the potential for these therapies to offer more effective management of C3G. The growth in awareness and diagnostic techniques, combined with the development of these therapies, will result in improved care and management for C3G patients.
- A rare form of kidney disease known as C3 Glomerulopathy (C3G) is associated with the buildup of protein deposits in kidney filtering tissues, leading to impaired kidney function and potential kidney failure.
- Science continues to advance in the realm of rare kidney diseases, with emerging therapies targeting proteins that contribute to C3G activity.
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