Focal segmental glomerulosclerosis (FSGS)

Focal segmental glomerulosclerosis (FSGS) is a disease in which scar tissue develops on the glomeruli, the small parts of the kidneys that filter waste from the blood. FSGS can be caused by a variety of conditions.

FSGS is a serious condition that can lead to kidney failure, which can only be treated with dialysis or kidney transplant. Treatment options for FSGS depend on the type you have.

Types of FSGS include:

• Primary FSGS. Many people diagnosed with FSGS have no known cause for their condition. This is called primary (idiopathic) FSGS.
• Secondary FSGS. Several factors, such as infection, drug toxicity, diseases including diabetes or sickle cell disease, obesity, and even other kidney diseases can cause secondary FSGS. Controlling or treating the underlying cause often slows ongoing kidney damage and might lead to improved kidney function over time.
• Genetic FSGS. This is a rare form of FSGS caused by genetic changes. It also is called familial FSGS. It’s suspected when several members of a family show signs of FSGS. Familial FSGS can also occur when neither parent has the disease but each one carries a copy of an altered gene that can be passed on to the next generation.
• Unknown FSGS. In some cases, the underlying cause of FSGS cannot be determined despite the evaluation of clinical symptoms and extensive testing.

Symptoms of focal segmental glomerulosclerosis (FSGS) might include:

• Swelling, called edema, in the legs and ankles, around the eyes and in other body parts.
• Weight gain from fluid buildup.
• Foamy urine from protein buildup, called proteinuria.

When to see a doctor

See a healthcare professional if you have any of the symptoms of FSGS.

Focal segmental glomerulosclerosis (FSGS) can be caused by a variety of conditions, such as diabetes, sickle cell disease, other kidney diseases and obesity. Infections and damage from illicit drugs, medicines or toxins also can cause it. Gene changes passed through families, called inherited gene changes, can cause a rare form of FSGS. Sometimes there’s no known cause.

Factors that can raise the risk of focal segmental glomerulosclerosis (FSGS) include:

• Medical conditions that can damage the kidneys. Certain diseases and conditions increase the risk of getting FSGS. These include diabetes, lupus, obesity and other kidney diseases.
• Certain infections. Infections that increase the risk of FSGS include HIV and hepatitis C.
• Gene changes. Certain genes passed through families can raise the risk of FSGS.

Focal segmental glomerulosclerosis (FSGS) may lead to other health concerns, also called complications, including:

• Nephrotic syndrome. This kidney condition causes the body to pass too much protein in the urine. Nephrotic syndrome raises the risk of other health conditions, such as blood clots and high blood pressure.
• Kidney failure. Damage to the kidneys that can’t be fixed causes the kidneys to stop working. The only treatments for kidney failure are dialysis or kidney transplant.

For possible focal segmental glomerulosclerosis (FSGS), your healthcare professional reviews your medical history and orders lab tests to see how well your kidneys work. Testing may include:

• Urine tests. These include a 24-hour urine collection that measures the amount of protein and other substances in the urine.
• Blood tests. A blood test called glomerular filtration rate measures how well the kidneys are getting rid of waste from the body.
• Kidney imaging. These tests are used to show kidney shape and size. They might include ultrasound and CT or MRI scans. Nuclear medicine studies also might be used.
• Kidney biopsy. A biopsy usually involves placing a needle through the skin to take a tiny sample from the kidney. The results of the biopsy can confirm a diagnosis of FSGS.

Treatment for focal segmental glomerulosclerosis (FSGS) depends on the type and the cause.

Medications

Depending on symptoms, medicines to treat FSGS might include:

• An angiotensin-converting enzyme (ACE) inhibitor or an angiotensin II receptor blocker (ARB). These can lower blood pressure and reduce protein in the urine.
• Medicines to lower cholesterol levels. People with FSGS often have high cholesterol.
• Medicines to help the body get rid of salt and water, called diuretics. These can improve blood pressure and swelling.
• Medicines to lower the body’s immune response. For primary FSGS, these medicines may stop the immune system from damaging the kidneys. These medicines include corticosteroids. They can have serious side effects, so they’re used with caution.

FSGS is a disease that may return. Because scarring in the glomeruli might be lifelong, you need to follow up with your healthcare team is to see how well your kidneys work.

For people who have kidney failure, treatments include dialysis and kidney transplant.

The following lifestyle changes can help keep the kidneys healthier:

• Don’t use medicines that can damage your kidneys. These include some pain relievers such as nonsteroidal anti-inflammatory drugs (NSAIDs). NSAIDS you can get without a prescription include ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve).
• Eat a healthy diet. To protect your kidneys and lower your blood pressure, limit salt and protein.
• Don’t smoke. If you need help quitting, talk with a member of your healthcare team.
• Stay at a healthy weight. Lose weight if you’re overweight.
• Be active on most days. Being active is good for your health. Ask your healthcare team what types of exercise and how much exercise you can do.

You may start by seeing your primary healthcare professional. Or you may be referred to a specialist in kidney conditions, called a nephrologist.

Here’s some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there’s anything you need to do before the appointment, such as not drinking or eating before having certain tests. This is called fasting.

Make a list of:

• Your symptoms, including any that seem unrelated to the reason for your appointment, and when they began.
• Key personal information, including major stresses, recent life changes and family medical history.
• All medicines, vitamins or other supplements you take, including doses.
• Questions to ask your healthcare team.

Take a family member or friend along, if possible, to help you remember the information you’re given.

For focal segmental glomerulosclerosis (FSGS), some basic questions to ask your healthcare professional include:

• What’s likely causing my symptoms?
• What are other possible causes for my symptoms?
• What tests do I need?
• Is my condition likely to go away or be long-lasting?
• What are my treatment choices?
• I have other health conditions. How can I best manage them together?
• Are there restrictions I need to follow?
• Should I see a specialist?
• Are there brochures or other printed material I can have? What websites do you think might be helpful?

Be sure to ask all the questions you have.

What to expect from your doctor

Your healthcare professional is likely to ask you questions, such as:

• Do your symptoms come and go or do you have them all the time?
• How severe are your symptoms?
• What, if anything, seems to make your symptoms better?
• What, if anything, seems to make your symptoms worse?