Focal Segmental Glomerulosclerosis (FSGS)

Many diseases and conditions can affect your kidney function by attacking and damaging the glomeruli, the tiny filtering units inside your kidney where blood is cleaned. These diseases and conditions are called glomerular diseases and can have many different causes. Focal Segmental glomerulosclerosis is a type of glomerular disease and describes scarring (sclerosis) in your kidney.  The scarring of FSGS only takes place in small sections of each glomerulus (filter), and only a limited number of glomeruli are damaged at first. Focal Segmental Glomerulosclerosis affects both children and adults. Males are affected slightly more often than females, and it occurs more frequently in African Americans

FSGS is not caused by a single disease. It can have many different causes. The scarring may happen because of an infection, or drug, or a disease that affects the entire body, like diabetes, HIV infection, sickle cell disease or lupus. FSGS can also be caused by another glomerular disease that you had before you got FSGS. FSGS has different types based on the cause.

Below are the types of FSGS:

• Primary FSGS: This type of FSGS means that the disease happened on its own without a known or obvious cause.
• Secondary FSGS: This type is caused by another disease or a drug. Examples include: viruses such as HIV or drugs such as anabolic steroids that some people use to speed up their muscle growth (these are different than steroids your doctor gives you for treatment).

Early stages may not cause any symptoms. You may only see some signs on your own, while others may be found by your healthcare provider.

Signs and symptoms of FSGs include:

• Swelling in body parts like your legs, ankles and around your eyes (called edema)
• Weight gain due to extra fluid building in your body
• Foamy urine caused by high protein levels in the urine (called proteinuria)
• High fat levels in the blood (high cholesterol)
• Low levels of protein in the blood

FSGS can cause nephrotic syndrome.

• Nephrotic Syndrome: A set of symptoms that happen together and affect your kidneys. These include:
  • Swelling in body parts like your legs, ankles, or around your eyes (edema)
  • Large amounts of protein in your urine (proteinuria)
  • Loss of protein in your blood
  • High levels of fat lipids in your blood (high cholesterol)
  • High blood pressure (in some cases)

If the condition is advanced, the symptoms may be like those of kidney failure. People may report fatigue, a poor appetite, headache, itchy skin, shortness of breath and/or nausea.

A blood test, urine test, and a kidney biopsy will determine if you have FSGS.

• Urine test: A urine test will help find protein and blood in your urine.
• Blood test: A blood test will help find levels of protein, cholesterol, and wastes in your blood.
• Glomerular filtration rate (GFR): A blood test will be done to know how well your kidneys are filtering the wastes from your body.
• Kidney biopsy:  In this test, a tiny piece of your kidney is removed with a special needle, and looked at under a microscope.
• Genetic testing: A genetic test may be done to see if you were born with genes that caused your kidney disease. This information may help your doctor decide what type of treatment is best for you.

The type of treatment you get depends on the cause. Everyone is different and your doctor will make a treatment plan that is right for your type of FSGS. Usually, treatments for FSGS include:

• Corticosteroids (often called “steroids”)
• Immunosuppressive drugs
• Plasmapheresis
• ACE inhibitors and ARBs
• Diuretics
• Diet change

Corticosteroids and immunosuppressive drugs: These medications are used to calm your immune system (your body’s defense system) and stop it from attacking your glomeruli.

ACE inhibitors and ARBs: These are blood pressure medications used to reduce protein loss and control blood pressure.

Diuretics: These medications help your body get rid of excess fluid and swelling. These can be used to lower your blood pressure too.

Diet changes:  Some diet changes may be needed, such as reducing salt (sodium) and protein in your food choices to lighten the load of wastes on the kidneys.

You should talk with your doctor about your condition because the progression of the disease depends on many factors.  FSGS is a chronic disease, because the scarred glomeruli cannot be repaired. Treatment can slow the process of kidney disease. Everyone is different in how they respond to treatment. Over time, some patients with FSGS gradually get worse until they reach kidney failure, If this occurs, they will need a kidney transplant or dialysis to stay alive. Some people respond well to treatment and may live with the disease for many years while being monitored for any signs of change.