Health Priority: FSGS

FSGS (Focal Segmental Glomerulosclerosis) is a rare kidney condition that is the leading cause of nephrotic syndrome (a kidney disorder) amongst African Americans. The term “focal segmental glomerulosclerosis” is defined as scarring or hardening (sclerosis) of parts (segmental) of some (focal) glomeruli (parts of the kidney) while other glomeruli remain unaffected. It is typically associated with edema (swelling), proteinuria (protein in urine, which is a way to measure kidney function), hypertension (high blood pressure), microscopic hematuria (blood in the urine), and renal insufficiency (poor kidney function). Recent studies suggest that either the incidence (new cases) of FSGS has increased or an increased number of biopsies of African American patients have made the diagnosis more common.

The condition can be the result of various issues:

• Virus infection
• Caused by medications 
• Linked to cancer 
• Altered structure or function of kidney after disease or surgery (i.e., diabetes, sickle cell disease, obesity)
• Nonspecific FSGS caused by kidney scarring 
• Genetic mutations 
• Without any known causes 

Recently, researchers have established a strong connection between abnormal variants in genes found in individuals of sub-Saharan African ancestry and the development of FSGS.

The disease can affect both children and adults with males tending to be slightly more susceptible. 

If you have been diagnosed with FSGS, here’s what you need to know now. 

Current Therapies: 

Currently, there are no medications approved specifically for the treatment of FSGS. Treatment involves a combination of drugs tailored to the cause of FSGS, a person’s age and other conditions (e.g. diabetes and obesity). The goal is to greatly reduce protein loss into the urine. 

This is achieved with drugs called angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor II blockers (ARBs). ACE inhibitors/ARBs are sometimes combined with immunosuppressants such as steroids (e.g. prednisone).

Clinical Trials: 

Clinical trials are another way that patients with FSGS can get treated for their disease. 

Currently, a biotech company called Retrophin is enrolling patients in the Phase 3 DUPLEX Study, to further understand how to help slow the decline of kidney function in patients with FSGS. 

Partner with your nephrologist and talk with family members about participating in the DUPLEX Study. Also, be sure to contact Patient Advocacy Groups such as NephCure, www.nephcure.org, for their specialized assistance. 

DID YOU KNOW…  

The following 10 regions have the highest incidence of kidney failure.  See where your state/region nets out.

RANK STATES/REGIONS

1. Alabama, Mississippi, Tennessee 
2. Georgia, North Carolina, South Carolina 
3. Southern California 
4. Texas 
5. Arkansas, Louisiana, Oklahoma 
6. Maryland, Virginia, W. Virginia, Washington, D.C. 
7. Northern California, Guam, Hawaii 
8. New York 
9. New Jersey, Puerto Rico, Virgin Islands
10. Illinois