Focal segmental glomerulosclerosis (FSGS)
Focal segmental glomerulosclerosis (FSGS) is a rare disease that causes scarring in your glomeruli, the tiny filters in your kidney. Each of your kidneys contain about 1 million glomeruli. The main job of glomeruli is to filter waste from your blood. They work just like a kitchen strainer. When blood circulates through glomeruli, the glomeruli keep the important parts your body needs. They strain out nutrients, minerals, extra fluid and waste products. The nutrients and minerals are sent back to your blood and the extra fluid and waste products become your pee. Kidney conditions that affect your glomeruli are called glomerular diseases. FSGS is just one of many types of glomerular diseases.
FSGS happens when small parts or sections of these tiny filters scar or harden (sclerosis). Of the damaged glomeruli, only some show scarring. When this scarring happens, it’s hard for your kidneys to act like a strainer. This can lead to kidney damage and, potentially, kidney failure. Treatment depends on what type of FSGS you have.
Here’s what the different parts of the name “focal segmental glomerulosclerosis’” mean:
- Focal: Some but not all glomeruli are affected.
- Segmental: Specific parts of the glomeruli have scarring.
- Sclerosis: Hardening.
The name ‘focal segmental glomerulosclerosis’ means hardening (sclerosis) of parts (segmental) of some (focal) glomeruli.
There are three types of focal segmental glomerulosclerosis:
- Primary FSGS: There’s no known reason or obvious cause for having FSGS.
- Secondary FSGS: This type occurs due to another disease or condition. Common causes could include infections, medications, drug use, sickle cell anemia or obesity. Controlling the underlying condition can often help treat secondary FSGS.
- Genetic (familial) FSGS: This type happens due to genetics. This means you can have FSGS if your biological parents have the genetic mutation for FSGS.
FSGS doesn’t always cause symptoms you might notice on your own. Healthcare providers often find signs or symptoms during a routine exam or when testing for other medical conditions. Some of these signs and symptoms include:
- Edema (swelling in your arms, legs or face).
- High cholesterol.
- Lower-than-average amounts of albumin (a protein in your blood).
- Proteinuria (abnormally high levels of protein in your pee).
- Sudden weight gain caused by extra fluids in your body.
- High blood pressure (hypertension).
When you have edema, proteinuria and low albumin, your provider may call it nephrotic syndrome. Nephrotic syndrome causes your kidneys to release too much protein in your pee.
There can be several different causes for FSGS. People who have primary FSGS often don’t have an obvious cause. Researchers believe that for unknown reasons, a protein in their blood damages part of the glomeruli.
Secondary FSGS happens when there’s too much blood flow to your glomeruli. Many factors can cause this, including:
- Diseases like sickle cell anemia, diabetes and lupus.
- Medications, such as interferons or bisphosphonates.
- Drugs such as heroin or anabolic steroids (substances used to enhance muscle growth).
- Infection, including viruses such as HIV.
- Having overweight or obesity.
- Other kidney conditions or having a congenital kidney condition.
The genetic form of FSGS happens when the APOL1 gene mutates during fetal development. This may happen more commonly in people with ancestry from West Africa.
FSGS can lead to high blood pressure and high cholesterol. In some cases, FSGS can lead to kidney failure. This condition can be life-threatening and requires emergency medical treatment. Seek immediate care if you have FSGS and experience any of these symptoms of kidney failure:
- Unmanaged high blood pressure.
- Anemia.
- Nausea, vomiting or loss of appetite.
- Swelling in your arms, legs or face.
Your healthcare provider will listen to your symptoms and take your medical history. Several types of kidney tests may help your provider diagnose FSGS. These tests can include:
- Blood test: Taking a sample of blood to measure levels of protein, waste and fat.
- Glomerular filtration rate (GFR): Checking a blood sample to measure how well your kidneys work.
- Urine test: Measuring the levels of blood and protein in your pee (urine).
However, providers can only definitively diagnose FSGS by doing a kidney biopsy. This is when a provider uses a needle to take a small sample of tissue from your kidney to look for signs of FSGS under a microscope.
Sometimes, healthcare providers use genetic testing to confirm a diagnosis of genetic FSGS. But because genetic tests can be expensive, and there are no known treatments for many forms of genetic FSGS, genetic testing isn’t common.
Treatment for FSGS depends on the type and cause, your age and whether you have other health conditions. The goal of treatment is to manage your symptoms to help you maintain a good quality of life and slow scarring so that it doesn’t lead to kidney failure.
In some people with secondary FSGS, treating the underlying condition may stop kidney damage from progressing. Your healthcare provider can’t repair damaged glomeruli, but over time, kidney function may improve.
Some treatments for FSGS include:
- Angiotensin-converting enzyme inhibitors (ACE-Is).
- Angiotensin receptor blockers (ARBs).
- Mineralocorticoid receptor blockers (MRBs).
- Antibiotics.
- Diuretics.
- Immunosuppressive drugs.
- Plasmapheresis.
- Corticosteroids.
Your healthcare provider may also recommend changes to your diet and lifestyle. This could include things like:
- Limiting how much sodium (salt) and protein you eat.
- Exercising daily.
- Quitting smoking. (Your healthcare provider can also give you resources to help with this.)
- Taking certain vitamins.
FSGS has no cure. The prognosis varies depending on the person. For some people, FSGS goes away on its own without treatment. For others, the disease continues for many years but doesn’t get worse.
Some people with FSGS develop kidney failure. They may need a kidney transplant or dialysis (a machine filters your blood instead of your kidneys). But many people with the disease live active, fulfilling lives. With regular checkups and blood tests, your provider will watch for any progression of the disease.
Some forms of FSGS you can’t prevent. But you can take steps to reduce your risk of FSGS by:
- Maintaining a weight that’s healthy for you.
- Treating conditions that are known causes of FSGS.
- Seeing your healthcare provider for yearly checkups to keep an eye on your health.
When should I see a healthcare provider about focal segmental glomerulosclerosis (FSGS)?
FSGS may not cause symptoms that you’ll notice on your own. Contact your healthcare provider if you experience unexplained swelling in your feet, legs or face. This is often the first symptom of FSGS. You may also see foamy or bubbly pee in the toilet after you pee, due to high amounts of protein in your urine.
What questions should I ask my doctor?
If you have FSGS, you may want to ask your provider:
- What caused FSGS?
- What’s the best treatment for me?
- How long will treatment last?
- What signs of complications should I look out for?
References
- Conversion disorder (functional neurological symptom disorder). In: Diagnostic and Statistical Manual of Mental Disorders DSM-5. 5th ed. American Psychiatric Association; 2013. https://dsm.psychiatryonline.org. Accessed Oct. 27, 2021.
- Aminoff MJ, et al.eds. Functional (psychogenic) neurologic disorders. In: Aminoff’s Neurology and General Medicine. 6th ed. Elsevier; 2021. https://www.clinicalkey.com. Accessed Oct. 27, 2021.
- AskMayoExpert. Functional neurologic disorder. Mayo Clinic. 2020.
- Functional neurologic disorder. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Functional-Neurologic-Disorder. Accessed Oct. 27, 2021.
- Gilmour GS, et al. Management of functional neurological disorder. Journal of Neurology. 2020; doi:10.1007/s00415-020-09772-w.
- Bennett K, et al. A practical review of functional neurological disorder (FND) for the general physician. Clinical Medicine. 2021; doi:10.7861/clinmed.2020-0987.
- Fobian AD, et al. A review of functional neurological symptom disorder etiology and the integrated etiological summary model. Journal of Psychiatry and Neuroscience. 2019; doi:10.1503/jpn.170190.
- O’Neal MA, et al. Treatment for patients with a functional neurological disorder (conversion disorder): An integrated approach. American Journal of Psychiatry. 2018; doi:10.1176/appi.ajp.2017.17040450.
- Medical review (expert opinion). Mayo Clinic. Nov. 16, 2021.
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