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 lead to kidney damage and even failure. Focal Segmental Glomerulosclerosis (FSGS) is a rare disease that attacks the kidney’s filtering system (glomeruli) causing severe 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 become scarred, just parts of them. So Focal Segmental Glomerulosclerosis means:
Focal = some
Segmental = sections
Glomerulo = of kidney filters
Sclerosis = are scarred
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 partial remission is significant. 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.
WHAT ARE THE FACTS OF FOCAL SEGMENTAL GLOMERULOSCLEROSIS?
More than 5400 patients are diagnosed with FSGS every year. This may be an underestimate because there are a limited number of biopsies performed and the number of FSGS cases is rising more than any other cause of Nephrotic Syndrome.
NephCure estimates that there are currently 19,306 people living with end-stage renal disease (ESRD) due to FSGS.
It is the second leading cause of kidney failure in children, in part because it is the most common cause of steroid-resistant Nephrotic Syndrome in children.
NephCure estimates that people of African ancestry are at a five times higher diagnosis rate of FSGS.
About half of FSGS patients who do not respond to steroids go into ESRD each year, requiring dialysis or transplantation.
Approximately 1,000 FSGS patients a year receive kidney transplants. However, within hours to weeks after a kidney transplant, FSGS returns in approximately 30-40% of patients.