Focal Segmental Glomerulosclerosis (FSGS): What You Need to Know

This article is for people who have focal segmental glomerulosclerosis (FSGS), or their care partners, or anyone who wants to learn more about FSGS. The goal of this activity is to educate patients on the importance of engaging with their doctor and healthcare team about managing and treating their FSGS.

You will learn about:

• What FSGS is

• How you and your doctor can recognize FSGS

• How FSGS is diagnosed and what tests your doctor may do

• Treatment options that are available

• What a clinical trial is

• Questions to ask your doctor

Focal segmental glomerulosclerosis -- or FSGS -- is a chronic (long-term) disease that happens in the part of the kidneys called the glomeruli, where waste products are filtered from your blood.

With FSGS, damage happens in the glomeruli and results in scarring, or sclerosis. At first, scarring only happens in small areas and only a limited number of glomeruli are damaged. But this scarring is serious -- scarred glomeruli cannot be repaired. Over time, FSGS can get worse and lead to permanent kidney damage and even kidney failure.  

FSGS has many different causes. Damage and scarring in the glomeruli can happen because of an infection, obesity, certain medicines, or certain conditions such as diabetes, human immunodeficiency virus (HIV) infection, sickle cell disease, lupus, or even another kidney disease. FSGS can also be inherited (passed through families) and caused by abnormal genes. And sometimes, no cause for FSGS can be identified at all.

There are 2 types of FSGS:

• Primary FSGS happens on its own without a known or obvious cause

• Secondary FSGS is caused by another condition or a medicine

When glomeruli are damaged, they can't filter blood properly and extra fluid, salt, and waste aren't removed from your body. As a result, you can get waste materials in your blood, and important products in your blood like protein can leak into your urine (proteinuria). This causes many of the symptoms of FSGS.

Some people with FSGS don't have symptoms. When they do appear, you may recognize certain ones. But others can be found by your doctor.

Symptoms you can recognize include:

• Swelling -- called edema -- from extra fluid. This usually happens in the legs, ankles, feet, hands, abdomen (belly), and around the eyes

• Weight gain from extra fluid

• Foamy urine due to protein in the urine

• Headache (from high blood pressure)

Your doctor or a nephrologist can do an exam and blood and urine tests to check for FSGS symptoms such as:

• High blood pressure

• High blood cholesterol

• Protein in your urine

• Low protein in your blood

• Abnormal levels of creatinine in your blood, a substance that shows how well your kidneys are working

These tests may be abnormal with many types of kidney disease. So to diagnose FSGS, you will also have a kidney biopsy where a tiny piece of kidney tissue is removed and examined. Your doctor may also recommend a genetic test to see if you have the genes that cause FSGS.

FSGS is a chronic (long-term) disease because the scarred glomeruli cannot be repaired. How your FSGS progresses may depend on several factors, including how severe your proteinuria is. But it's important to know that proper treatment can slow down your kidney disease.

FSGS can cause a serious condition called nephrotic syndrome. Over time, FSGS and nephrotic syndrome can worsen, and even lead to kidney failure. This means that your kidneys are working at less than 15% of their normal function. If this happens, you will need a kidney transplant or dialysis to stay alive.

The goals of treating FSGS are to stop protein from spilling into the urine, control symptoms, and prevent kidney failure. Which treatment your doctor recommends will depend on the type and cause of your FSGS, as well as your age, test results, and symptoms.

Treatment may include:

• Corticosteroids and immunosuppressive medicines to decrease your immune system's function and protein spilling into your urine. In some people with FSGS, an active immune system may cause damage to the glomeruli

• Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) to control blood pressure and reduce protein in the urine

• Diuretics (water pills) to help get rid of excess fluid, decrease swelling, and lower blood pressure

Your doctor will work with you to find the right treatment plan for your FSGS. Most people with FSGS will see a kidney specialist, or nephrologist.

As part of this plan, your doctor may recommend a special diet to help reduce swelling and the work your kidneys have to do. It will be a diet low in salt (sodium) and your doctor may also adjust the amounts of potassium, phosphorus, and protein.

Everyone responds to treatment differently, so talk to your doctor about the best way to manage your FSGS. You can also ask if participating in a clinical trial might be an option for you.

During a clinical trial, researchers study patients with a specific disease or condition who are using a new medical treatment.

There are clinical trials studying new treatments for FSGS. Information can be found on the internet and on the websites listed in the Additional Materials section at the end of this activity. You can also ask your doctor or nephrologist if participating in a clinical trial might be right for you.

Maintaining a healthy lifestyle can help you manage your FSGS and keep your kidneys functioning.

Ways to do this include: 

• Quit smoking

• Take medicines as directed and tell your doctor about any side effects

• Talk to your doctor or pharmacist before taking new medicines, including any you get without a prescription

• Control your blood pressure

• Follow the diet recommended by your doctor and drink plenty of water (if you are not on any fluid restrictions)

• Limit or stop drinking alcohol

• Stay physically active

• Get enough sleep -- 7 to 8 hours a night

Visit your doctor or nephrologist regularly so they can check how your treatment is working and your FSGS and kidney function.

Questions to ask can include:

• Do you specialize in treating FSGS?

• What can I do to help manage my FSGS and its symptoms?

• What treatments are available and what are their side effects?

• What changes should I make to my lifestyle?

• What type of diet should I eat and exercise should I do?

• How can I find out about clinical trials for FSGS and if participating in one might be right for me?

Congratulations!

You have successfully completed the program: Focal Segmental Glomerulosclerosis (FSGS): What You Need to Know

View Additional Materials

View Additional Materials on this topic that you may find useful:

Focal segmental glomerulosclerosis -- NIH

Focal Segmental Glomerulosclerosis (FSGS) -- NephCure Kidney International

Focal Segmental Glomerulosclerosis (FSGS) -- National Kidney Foundation

Focal Segmental Glomerulosclerosis -- NORD

Ongoing Clinical Trials

ClinicalTrials.gov