Nephrology

IgAN & C3G

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Best Supportive Care for Patients With C3 Glomerulopathy

patient care perspectives by Richard Lafayette, MD, FACP
Overview

An optimal targeted treatment for patients with C3 glomerulopathy (C3G) has not been established; however, supportive therapy is an important initial step in the treatment of these patients. Best supportive care practices include lifestyle and dietary modifications and treatments that reduce blood pressure and proteinuria.

Expert Commentary
“We start with generic supportive therapy to try to keep the kidneys healthy. This includes a healthy lifestyle, which means regular exercise and no smoking or excessive alcohol intake. We want patients to try to achieve, or at least approach, a more ideal body weight because being overweight stresses the kidneys and can cause more rapid disease progression.”
— Richard Lafayette, MD, FACP

C3G is a very rare disease, but it manifests very differently patient by patient. Our approach to supportive care really depends on the individual and their disease symptoms. Across kidney disease, we make broad efforts to first educate patients about their disease so that they understand what is going on, emphasizing that they have a role in their own disease management. I think that it is really important for patients to feel a partnership in their self-care.

 

We start with generic supportive therapy to try to keep the kidneys healthy. This includes a healthy lifestyle, which means regular exercise and no smoking or excessive alcohol intake. We want patients to try to achieve, or at least approach, a more ideal body weight because being overweight stresses the kidneys and can cause more rapid disease progression. Americans tend to have diets that are very high in sodium and protein, and diets containing lower amounts of salt and protein may be beneficial. So, we spend a lot of time working with the patient, often sending them to a dietician to help them understand the numbers that underlie recommendations and what those numbers mean in terms of food.

 

In addition, we do not want patients to do things independently that might hurt their kidneys. Thus, they are all counseled to avoid NSAIDs. If a patient requires medications, we need to discuss those medications with their physician to make sure that they are completely safe for their kidneys.

 

Then you get to more interventional therapy based on patient risk. For patients with significant proteinuria, which is often a manifestation of C3G, we want their blood pressure to be perfect, which generally means under 120/80 mm Hg. Aggressive control of blood pressure is important, and, again, if a patient has proteinuria, we want to be aggressive with agents that can lower proteinuria. That means using RAAS inhibitors (at maximally tolerated doses) to try to reduce the proteinuria and help with blood pressure control, especially when their diet is low in salt.

 

Emerging supportive therapies are coming along in development. They are not ready for prime time yet, but I think that agents that block the endothelin system or the RAAS may help in patients with significant proteinuria. Further, there is more and more attention being paid to SGLT2 inhibitors, which may be valuable for patients with proteinuric nephropathies such as C3G. As supportive care is being optimized, considerations will be made for disease-specific therapy.

 

There are other important considerations for patients with kidney disease. For example, it is often younger patients who are being diagnosed, so these individuals may be thinking about family planning. We want to make sure that women in particular consider contraception and whether pregnancy could cause glomerular complications.

 

Getting involved in support groups is often very helpful. For C3G, there is no large support organization, but there are national organizations for rare diseases, such as NephCure, which focuses on protein-spilling kidney disease, as well as other national organizations, including the American Association of Kidney Patients and the National Kidney Foundation. Local organizations, local chapters of the National Kidney Foundation, and support groups for family and friends can also help provide support for patients. In addition, patient reliance on peer groups, where they may meet other patients with their disease who have benefited from working on their diet, exercise, and medication compliance, is very important. Finally, it is also important for patients to understand that C3G is a chronic disease that needs to be managed and has a risk of progressing to the need for dialysis or kidney transplant. Developing long-term relationships with good communication is an important key to success.

References

Campbell ZC, Dawson JK, Kirkendall SM, et al. Interventions for improving health literacy in people with chronic kidney disease. Cochrane Database Syst Rev. 2022;12(12):CD012026. doi:10.1002/14651858.CD012026.pub2

 

Chen Y, Dabbas W, Gangemi A, et al. Obesity management and chronic kidney disease. Semin Nephrol. 2021;41(4):392-402. doi:10.1016/j.semnephrol.2021.06.010

 

Gonzalez Suarez ML, Kattah A, Grande JP, Garovic V. Renal disorders in pregnancy: core curriculum 2019. Am J Kidney Dis. 2019;73(1):119-130. Published correction appears in Am J Kidney Dis. 2019;73(6):897.

 

Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021;100(4S):S1-S276. doi:10.1016/j.kint.2021.05.021

 

MacLaughlin HL, Friedman AN, Ikizler TA. Nutrition in kidney disease: core curriculum 2022. Am J Kidney Dis. 2022;79(3):437-449. doi:10.1053/j.ajkd.2021.05.024

 

Noris M, Remuzzi G. C3G and Ig-MPGN—treatment standard. Nephrol Dial Transplant. 2024;39(2):202-214. doi:10.1093/ndt/gfad182

 

Pugh D, Gallacher PJ, Dhaun N. Management of hypertension in chronic kidney disease. Drugs. 2019;79(4):365-379. Published correction appears in Drugs. 2020;80(13):1381.

Richard Lafayette, MD, FACP

Professor of Medicine (Nephrology)
Director
Stanford Glomerular Disease Center
Stanford University Medical Center
Stanford, CA

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