National Organization for Rare Diseases
Focal segmental glomerulosclerosis is a rare and complex form of kidney disease. Learn what it is and what it could mean for your overall health.
Focal segmental glomerulosclerosis (FSGS) is a kidney disease that affects approximately seven (7) out of a million people, but it accounts for up to 20% of all people in the U.S. who experience kidney failure. Though the disease is rare, if you are one of the few people who have it, it’s important to know how FSGS affects you and what can be done to treat it.*
FSGS damages your kidneys by scarring the tiny filtering units (glomeruli) in your kidneys that clean your blood of wastes. This scarring, or hardening, of tissue is called sclerosis, and in FSGS, it affects only parts (segmental) of selected (focal) glomeruli while others remain healthy.
The damage from scarring, visible under a microscope, affects only a few glomeruli in the beginning, and a variety of factors determine whether the disease progresses slowly or quickly to kidney failure.
There have been several attempts to classify the different types of FSGS, the most straightforward being based on each type’s underlying cause.
FSGS can affect both adults and children, but factors linked to higher risk and faster kidney function decline include being:
Research supports a strong association between FSGS and abnormal variants of the APOL1 gene, which occurs in individuals of sub-Saharan African ancestry.
Signs and symptoms of FSGS are directly related to the degree of damage to your kidneys. Some people are asymptomatic, while others rapidly progress to kidney failure. While certain signs and symptoms are noticeable, others can only be detected through lab tests of your blood and urine, and other diagnostic devices.
Some common signs and symptoms include:
A diagnosis of FSGS is based upon a:
Several other kidney diseases present with similar symptoms to those of FSGS. A few of those diseases include:
Due to this widespread overlap of symptoms, a diagnosis of FSGS is typically confirmed by a kidney biopsy.
The primary goals of treating FSGS are to decrease proteinuria, stabilize or improve kidney function, and manage symptoms. The choice of treatment methods is dependent upon multiple factors, such as:
Medication is the first-line treatment. Patients with primary FSGS may be treated with:
For patients with virus-associated FSGS, the main goal of treatment is to eradicate the underlying infection.
Your doctor will also likely suggest lifestyle changes such as:
For people whose kidneys no longer function, dialysis or kidney transplantation are necessary to lengthen life beyond that offered by palliative care. In the case of a transplant, there is a risk that FSGS will recur in the transplanted kidney, particularly for patients with primary FSGS. If this occurs, further medications and procedures may be added to your treatment and care plan.
More information on FSGS can be found on the National Kidney Foundation (NKF) and Mayo Clinic websites.
*National Organization for Rare Diseases. (2018, Nov. 21). What Is Focal Segmental Glomerulosclerosis? https://rarediseases.org/rare-diseases/focal-segmental-glomerulosclerosis/
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