My doctor tells me I have focal glomerulosclerosis. What does this mean?

Focal glomerulosclerosis describes scarring in the kidney. There are more than 20 types of glomerular diseases. These are what the words mean:

• “Glomerulo-” refers to the clusters of tiny blood vessels in the kidneys that filter waste from the blood.
• “Sclerosis” refers to scarring.
• “Focal” means that only some of kidneys’ glomeruli have been damaged, while some are still normal.
• If you have focal segmental glomerulosclerosis (FSGS), it means the scarring is more limited.

Glomerulosclerosis affects both children and adults. Males are affected slightly more often than females, and it occurs more frequently in African Americans.

What causes glomerulosclerosis?

Glomerulosclerosis is not caused by a single disease. It has several different causes. The scarring may be the result of an infection, or drug toxicity, or a disease that affects the entire body, like diabetes, HIV infection, sickle cell disease or lupus disease. Sometimes, there is no apparent associated disease or cause. There may be a genetic component in some people.

People with these conditions may develop glomerulosclerosis:

• Glomerulonephritis, an inflammation of the glomeruli.
• Reflux nephropathy (backward flow of the urine up to the kidney).
• Kidney-harming chemicals or medicines that damage the glomeruli.

What are the signs and symptoms?

Early stages of glomerulosclerosis may not cause any symptoms.

Your doctor may suspect kidney disease because you develop high blood pressure or show protein in your urine.

If very large amounts of protein are lost in the urine, swelling of the ankles and eyelids may occur, because the body retains water (edema). This may also cause rapid weight gain and a make high blood pressure harder to control.

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

What tests do I need?

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

Blood test: Your kidney function will be evaluated using the glomerular filtration rate (GFR). Your GFR number is calculated from a math equation that considers the results from a blood test that combines your blood creatinine level, your age, gender and race. People with glomerulosclerosis can have a low GFR, as their kidney damage progresses and kidney function decreases.

Urine tests: Your doctors will order urine tests for protein or red blood cells. Having large amounts of protein appearing in the urine is common in glomerulosclerosis. Blood in the urine is a warning sign of kidney disease.

Biopsy: Having a low GFR and protein in the urine is not proof of glomerulosclerosis, though. Since these are associated with other kidney conditions, a kidney biopsy is needed to diagnose glomerulosclerosis. In this procedure, a needle is used to take a tiny sample of the kidney to examine with a microscope. The diagnosis is made from the signs of scarring seen on the kidney tissue sample.

Glomerulosclerosis can cause nephrotic syndrome. Nephrotic syndrome means very high levels of protein in the urine, low blood protein levels, high cholesterol levels, and swelling.

How are focal glomerulosclerosis and FSGS treated?

Treatments for FSGS include corticosteroids (often called “steroids”) and other immunosuppressive drugs (such as cyclosporine). These medications are used to decrease proteinuria and improve kidney function. Urine is tested for protein frequently during treatment, because having less urinary protein is a sign that the treatment is working.

Another important part of treatment is to control blood pressure and blood cholesterol levels, factors that add to the risk of complications from kidney disease. Certain blood pressure medicines, called ACE (angiotensin converting enzyme) inhibitors and ARBs (angiotensin receptor blockers), are used to reduce the protein loss and blood pressure. Diuretics are medicines that help the body get rid of excess fluid and swelling. These can be used to lower your blood pressure too.

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

What can I expect? Will treatment cure glomerulosclerosis?

Glomerulosclerosis is a chronic disease, because the scarred glomeruli cannot be repaired. Treatment can slow the progression of kidney disease. Everyone is different in how they respond to treatment, any complications, and the rate of disease progression. Over time, some patients with glomerulosclerosis gradually get worse until they reach kidney failure, If this occurs, they will need a kidney transplant or dialysis (hemodialysis or peritoneal 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.

Should I get ready for a kidney transplant?

You should talk with your doctor about your condition, because the progression of the disease depends on many factors. The underlying cause, your age, and your overall health affect your outcome. Patients who develop kidney failure from glomerulosclerosis can be good candidates for kidney transplants. You should know that glomerulosclerosis can recur in the transplanted kidney, but that should not discourage you from seeking a transplant.

What research is being done in this condition?

Researchers are trying to answer the many unanswered questions about this disease. They are now better able to explain what happens in the kidney during glomerulosclerosis. New drug treatments are in clinical trials. Genetic and molecular research is making progress in understanding this condition and suggesting how it could be more effectively treated.

Glomerulonephritis