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FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS)

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FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS)

Focal segmental glomerulosclerosis is a rare condition in which scar tissues develop on the glomeruli, which are tiny units within the body that filters blood.

  • Each kidney is made up of approximately 1 million glomeruli.
  • Glomeruli remove extra fluid, electrolytes, and waste from the bloodstream.
  • If the glomeruli are damaged or scarred, it can disrupt the proper functioning of the kidney leaving it unable to remove waste and excess fluids efficiently.
  • As a result, dangerous levels of fluid, electrolytes and waste can build up in the body.
  • This can lead to complication such as permanent kidney damage and eventually kidney failure
  • The word “FOCAL” is added because the scarring only takes place in some of the glomeruli filters.
  • This condition affects both children and adults but it is more common in adults especially in those older than 45 years.
  • It is also more prevalent in men than women, and people of African American and Asian descent.

CAUSES

FSGS has different type based on the cause. They include:

  • Primary FSGS. In this type, the condition happens on its own without no known cause. This is also called idiopathic FSGS.
  • Secondary FSGS. This type is caused by certain factors such as infections, drug toxicity such as heroin and pamidronate, diseases such as diabetes mellitus, sickle cell disease, obesity, chronic pyelonephritis, congenital kidney defects, HIV, or autoimmune disorders such as Lupus.
  • Some forms are genetic. Some genes associated with this condition include ACTN4, TRCP6, CD2AP, INF2, and mutations in the gene NPHS2 which encodes for the protein called podocin.

SYMPTOMS

Many people with FSGS do not have symptoms at all. However, if symptoms are present, they may include:

  • Swelling in some body parts, noticeable around the eyes, legs, ankles, abdomen (called edema)
  • Foamy urine caused by high amounts of protein ‘spilling’ into the urine (proteinuria)
  • Weight gain
  • High cholesterol in some cases
  • High blood pressure

DIAGNOSIS AND TREATMENT

To make a diagnosis, the doctor will carry out:

Urine tests to help find protein and blood in the urine

Blood tests to help find the levels of cholesterol, proteins, and wastes in the blood.

Glomerular filtration rate to detect how well your kidney is filtering waste from your body.

Kidney biopsy where a sample tissue is taken from the kidney for testing in the laboratory.

A genetic test may be done to see if there are mutations in your gene that may cause kidney problems.

TREATMENT

The aim of treatment is to slow down the process of kidney failure because the scarred glomeruli cannot be repaired.

Treatment options may include

  • Corticosteroids called prednisone to try and control proteinuria
  • Immunosuppressive drugs
  • Diuretics and low salt diet to help relieve edema
  • ACE inhibitors
  • Anticoagulants to prevent blood clots
  • A healthy diet that includes fruits and vegetables, low in saturated fats and cholesterol, and correct amounts of fluid and protein intake.
  • Exercising
  • Smoking cessation

Everyone is different in their response to treatment. While some patient responds well to treatment and may live with the conditions for many years while being monitored for any changes, some patient can get worse until they reach kidney failure, and may need dialysis or kidney transplant to stay alive.