What is Focal Segmental Glomerulosclerosis?
Each person has two kidneys in their lower back. Each kidney is made up of approximately one million tiny filters called “glomeruli.” Much as a coffee filter keeps coffee grounds in, glomeruli keep valuable cells and protein in the blood. When glomeruli become damaged, proteins begin leaking into the urine (proteinuria). Proteinuria causes fluid to accumulate in the body, and prolonged leakage can lea d to kidney damage and even failure. Focal Segmental Glomerulosclerosis (FSGS) is a rare disease that attacks the kidney’s filtering system (glomeruli) causing serious scarring. FSGS is one of the causes of a serious condition known as Nephrotic Syndrome.
What does Focal Segmental Glomerulosclerosis mean?
Sclerosis means “Scarring.” There are millions of tiny, microscopic filters in the kidneys called “glomeruli.” They are filters much like a sieve you might find in your kitchen, and they filter the blood, taking out the water-like part that becomes urine and leaving the protein in the blood. If the glomeruli become scarred, then they stop doing a good job filtering the blood and protein starts leaking into the urine instead of staying in the blood. The word “focal” is added because in FSGS, only some of the glomeruli filters become scarred. “Segmental” means that only some sections of the glomerulus becomes scarred, just parts of them. So Focal Segmental Glomerulosclerosis means:
• Focal = some
• Segmental = sections
• Glomerulo = of kidney filters
• Sclerosis = are scarred
How is Focal Segmental Glomerulosclerosis diagnosed?
FSGS is diagnosed with renal biopsy (when doctors examine a tiny portion of the kidney tissue), however, because only some sections of the glomeruli are affected, the biopsy can sometimes be inconclusive.
What causes Focal Segmental Glomerulosclerosis?
FSGS is “idiopathic,” which means it arises without a known cause, so researchers are actively trying to learn more.
What are some symptoms of Focal Segmental Glomerulosclerosis?
FSGS causes inefficient filtering of wastes from the blood which in return causes the following symptoms:
• Proteinuria – Large amounts of protein “spilling” into the urine
• Edema – Swelling in parts of the body, most noticeable around the eyes, hands and feet, that can become painful
• Hypertension – High blood pressure
• Hypoproteinemia – Low blood protein
• Hypercholesterolemia – High level of cholesterol in the blood
• Proteinuria Treatment aims to decrease the amount of protein lost in the urine
• The less protein in the urine, the better the patient will do
• Even a partial remission is important
• Many adults can achieve a complete or partial remission with immune suppressing medications
How is Focal Segmental Glomerulosclerosis treated?
Currently there are no FDA approved treatments, but usually a steroid called prednisone or prednisolone,is given to control proteinuria.
Your nephrologist may also recommend:
• Medications that suppress your immune system
• Diuretics and low salt diet help to control edema
• A medication that blocks a hormone system called the renin angiotensin system (ACE inhibitor or ARB) to control blood pressure or lower urine protein
• Anticoagulants to prevent blood clots
• Statins to lower the cholesterol level
• Maintaining a healthy diet: Correct amounts of protein and fluid intake according to your nephrologist’s recommendations. A healthy diet consists of low salt with emphasis on fruits and vegetables, low in saturated fat and cholesterol. A low salt diet may help with swelling in the hands and legs. For more guidance on suggested foods visit the Children’s Hospital web site.
• Not smoking
What are the facts of Focal Segmental Glomerulosclerosis?
More than 5400 patients are diagnosed with FSGS every year, however, this is considered an underestimate because: a limited number of biopsies are performed, and the number of FSGS cases are rising more than any other cause of Nephrotic Syndrome.1 NephCure estimates that there are currently 19,306 people living with ESRD due to FSGS2 , in part because it is the most common cause of steroid resistant Nephrotic Syndrome in children,3 and it is the second leading cause of kidney failure in children.4 NephCure estimates that people of African ancestry are at a five times higher diagnosis rate of FSGS.5 About half of FSGS patients who do not respond to steroids go into ESRD each year, requiring dialysis or transplantation.6 Approximately 1,000 FSGS patients a year receive kidney transplants7 however, within hours to weeks after a kidney transplant, FSGS returns in approximately 30-40% of patients.