Treatment for C3 glomerulopathy involves a combination of medications and lifestyle changes to manage the symptoms and prevent or slow kidney damage. There isn’t a cure for this rare disease, but targeted treatment can help.
You’ll most likely work with a team of health care professionals to keep your condition as well-controlled as possible.
Here’s what you should know about your health care team and how they may help you manage this disease.
Meet the Members of Your Team
The following health care professionals can help you manage your C3 glomerulopathy:
Primary care provider (PCP). They monitor your kidney health at routine medical visits, see you when you’re sick, and help you manage related conditions like high blood pressure.
Nephrologist. This is a doctor who specializes in kidney diseases. They will monitor your kidney function and blood pressure.
Cardiologist. This specialist can keep tabs on your blood pressure, blood cholesterol, and other heart risks and adjust heart-related medications as needed.
Registered dietitian. Many people with C3 glomerulopathy need to be on a low-sodium, low-protein diet. A dietitian can help you create an eating plan.
Pharmacist. They fill prescriptions, educate you about your medicines, make sure none of them will cause dangerous interactions, and sometimes recommend medication changes to your doctors if needed.
Social worker. They can help you navigate all the different types of care you’ll need. They’ll also help you cope with the emotional toll of this condition and refer you for more help if you need it.
Putting Together a Care Plan
Your health care team will come up with a plan of care depending on whether your disease is mild, moderate, or severe. These are some of the treatment plans your team might recommend:
Mild disease. Your C3 glomerulopathy is mild if you have less than 1.5 grams of protein in your urine each day and normal kidney function. Your health care team will usually recommend:
- Diet changes. A low-sodium, low-protein diet puts less strain on your kidneys. That’s usually less than 2 grams of sodium a day and less than 0.8 grams/kilogram of protein a day (that means about 54 grams of protein for a 150-pound person).
- Blood pressure-lowering medications. People with C3 glomerulopathy usually get blood pressure drugs called ACE inhibitors or ARBs.
- Cholesterol medications. Your doctor will want to check your cholesterol level frequently. If it’s elevated, you will get a cholesterol-lowering drug called a statin to treat it.
Moderate to severe disease. If you have more than 1.5 grams of protein in your urine every day, or your kidney function has declined, you will need more aggressive treatment.
Your doctor may suggest a complement inhibitor approved to treat C3 glomerulopathy such as:
- Iptacopan (Fabhalta), research shows that it may slow the progression of your disease.
- Pegcetacoplan (Empaveli) which works to lower protein in your urine and slow kidney damage.
These targeted treatments can help
Other treatments for C3 glomerulopathy
Your doctor may also suggest using drugs such as:
- Azathioprine
- Cyclophosphamide
- Eculizumab (Soliris)
- Glucocorticoids (corticosteroids, TRF-budesonide)
- Mycophenolate mofetil (MMF)
- Rituximab
- Sirolimus
- Tacrolimus
The medicines can give mixed results, but research suggests combining MMF and steroids may work best. In studies, people had lower rates of remission and end-stage kidney disease (ESKD) after taking these two medications for about 4 years than those who took other immunosuppressant drugs or none at all.
When to Switch Treatment Plans
Not everyone with C3 glomerulopathy gets better on immunosuppressants. If you don’t benefit from this medication, your health care team may recommend a drug called eculizumab. It blocks the part of the immune system that harms people with C3 glomerulopathy.
Your doctor may do a blood test to check your levels of a substance called soluble C5b-9 before they start you on this medication. If your levels are high, there’s a good chance that you will do well on this drug. If you do respond, you’ll most likely see improvements in your condition within 3 months.
Some people with C3 glomerulopathy who have certain genetic mutations linked to the disease may get a plasma infusion or a plasma exchange. Plasma is the fluid part of your blood. It contains proteins.
During a plasma infusion, you receive plasma through a tube inserted into a vein. This new plasma adds proteins to your blood that may help prevent C3 glomerulopathy. Plasma exchanges remove harmful proteins and replace them with healthy ones.
How to Handle Complications
If you have C3 glomerulopathy, you have a very high chance of getting end-stage kidney failure. An estimated 70% of children and up to 50% of adults with C3 glomerulopathy end up with it within 10 years of their diagnosis.
When that happens, you’ll need to go on dialysis or have a kidney transplant. Unfortunately, more than half the time, C3 glomerulopathy comes back after a kidney transplant. That’s because the problem starts in your immune system, not in your kidneys.
Treating C3 glomerulopathy complications
Sometimes, people with C3 glomerulopathy lose kidney function fast over a very short time – sometimes just days or weeks. This is a condition called rapidly progressive glomerulonephritis. Doctors usually treat this in the hospital with glucocorticoids and cyclophosphamide or MMF to try to suppress inflammation.
If you have the dense deposit disease type of C3 glomerulopathy, your health care team may recommend that you see an eye doctor regularly. People with this form of C3 glomerulopathy are more likely to have drusen, which are protein deposits in the eyes.
These can hurt your vision, so it’s important to get a diagnosis and treatment. People with these protein deposits often respond to drugs for age-related macular degeneration, which also involves drusen.
Treatments on the Horizon
Clinical trials are underway to look at new treatments for C3 glomerulopathy. A few treatments currently in trials are:
Avacopan. This complement inhibitor is already FDA-approved to treat other conditions. The medicine allows your immune system to fight off infection, but still blocks the germ-fighting white blood cells from causing kidney inflammation.
But recent FDA reports suggest avacopan can raise your risk for serious liver injury, drug-induced liver injury (DILI), vanishing bile duct syndrome (VBDS), and potentially death. VBDS is a rare liver condition where avacopan slows the flow of bile in your liver, eventually destroying the ducts in your liver until they disappear.
If you’re taking avacopan, contact your doctor as soon as possible. They’ll help you decide whether continuing treatment or switching medicines is best for your situation.
Daratumumab. This drug has worked well in people with multiple myeloma, a blood cancer that involves abnormal protein production. Researchers believe it could help some people with C3 glomerulopathy, too, since the disease can also start with a protein problem for some people.
Clinical trial. Ask your doctor if a clinical trial might be right for you and how you can enroll.
It may seem frustrating that there’s no immediate cure for C3 glomerulopathy. But your health care team can work with you to create the treatment plan that will work best for you.