Responsum for CKD

{{user.displayName ? user.displayName : user.userName}}
{{ user.userType }}
Welcome to

Responsum for CKD

Already a member?

Sign in   
Do you or someone you know have CKD?

Become part of the foremost online community!

Sign Up Now

Or, download the Responsum for CKD app on your phone

National Organization for Rare Diseases

National Organization for Rare Diseases

Focal Segmental Glomerulosclerosis (FSGS): What Is It?

Focal Segmental Glomerulosclerosis (FSGS): What Is It?

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.


Published on {{articlecontent.article.datePublished | formatDate:"MM/dd/yyyy":"UTC"}}
Last reviewed on {{articlecontent.article.lastReviewedDate | formatDate:"MM/dd/yyyy":"UTC"}}

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.*

What is FSGS?

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. 

What are the causes of FSGS?

There have been several attempts to classify the different types of FSGS, the most straightforward being based on each type’s underlying cause.

  • Primary FSGS. Also known as idiopathic FSGS, this is the most common form. Idiopathic diseases develop with no known or obvious cause.
  • Secondary FSGS. Also called adaptive or postadaptive, this results from:
    • Medical conditions that stress or strain the glomeruli, including:
      • obesity, diabetes, and high blood pressure 
      • lupus 
      • sickle cell disease 
      • congenital kidney defects 
    • The healing process from a previous kidney injury 
    • The direct toxic effect of certain drugs (such as anabolic steroids and heroin) or viruses (such as HIV, hepatitis C, and Epstein-Barr). 
  • Genetic FSGS. This is caused by a gene abnormality that either damages the glomeruli directly or creates a predisposition for that damage to occur. 

Who is at risk for FSGS?

FSGS can affect both adults and children, but factors linked to higher risk and faster kidney function decline include being:

  • Male
  • Aged 45 or older 
  • African American

Research supports a strong association between FSGS and abnormal variants of the APOL1 gene, which occurs in individuals of sub-Saharan African ancestry.

What are the signs and symptoms of FSGS?

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:

  • Fluid retention and swelling in the feet, ankles, hands, and face 
  • Foamy urine 
  • Weakness and fatigue 
  • High blood pressure 
  • Loss of appetite 
  • Unintended weight gain 
  • Excess amounts of protein in your urine
  • High cholesterol levels
  • Increased tendency to form blood clots

How is FSGS diagnosed?

A diagnosis of FSGS is based upon a:

  • Detailed patient history 
  • Clinical evaluation to identify characteristic symptoms
  • Variety of specialized tests, including blood tests, urine tests, and molecular genetic testing 

Several other kidney diseases present with similar symptoms to those of FSGS. A few of those diseases include:

  • Membranous nephropathy 
  • Amyloidosis 
  • C3 glomerulopathy 
  • Alport Syndrome 
  • Lupus nephritis 
  • IgA nephropathy

Due to this widespread overlap of symptoms, a diagnosis of FSGS is typically confirmed by a kidney biopsy.

How is FSGS treated?

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:

  • Underlying cause(s)
  • Extent of kidney function decline
  • Presence or absence of certain symptoms
  • Patient’s age and overall health
  • Potential risks and benefits of a given treatment
  • Patient’s preference

Medication is the first-line treatment. Patients with primary FSGS may be treated with:

  • Steroids and/or other immunosuppressants
  • Blood pressure drugs without immune system suppression
  • Water pills (diuretics) to relieve swelling 
  • Blood-thinners to help prevent blood clots 
  • Statins to lower cholesterol levels and blood pressure

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:

  • A low-salt, low-protein diet
  • Regular exercise
  • Smoking cessation
  • Vitamin supplements
  • Stress management 

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/

Source: {{articlecontent.article.sourceName}}

 

Join the CKD Community

Receive daily updated expert-reviewed article summaries. Everything you need to know from discoveries, treatments, and living tips!

Already a Responsum member?

Available for Apple iOS and Android