Focal and segmental glomerulosclerosis (FSGS) is a rare kidney disease where the tiny filters that clean the blood (glomeruli) become damaged and leak protein into your urine. The disease gets its name because the damage (scarring or sclerosis) that you see on a kidney biopsy sample is on some but not all glomeruli (focal) and shows up in sections (segmental).
FSGS is one of the most common causes of nephrotic syndrome in adults in Canada. It is less common in children.
What causes FSGS?
FSGS can be:
- Primary: It happens on its own without a known reason. It’s often related to problems in the immune system.
- Secondary: It’s likely related to one of your medicines, a drug exposure, another health condition, such as low birth weight, obesity, or a viral infection, or in some cases may be genetic (you inherited it from your family).
What are the symptoms of FSGS?
Symptoms of FSGS include:
- a lot of protein in the urine (proteinuria) This can make the urine look frothy.
- low levels of blood protein (hypoalbuminemia)
- swelling (edema) This happens when your body holds on to extra salt and water. You may notice swelling in the face, eyes, belly, hands, and feet, and it can cause weight gain.
- high cholesterol
Children and adults may also have other symptoms. Adults may feel tired and have trouble focusing. Children may not feel hungry (poor appetite), they may feel more cranky and tired, or pee less than usual. Talk to your healthcare provider about how you or your child are feeling to help find out if these other symptoms may be related to GN.
What is the treatment for FSGS?
The first step in managing FSGS is to find out if there is a medicine or other health condition causing the FSGS. If there is, the treatment would be to stop the taking the medicine that’s causing the problem or treat the other health condition.
For primary FSGS, the first treatment is usually prednisone, a corticosteroid medicine. There are other treatments if prednisone isn’t working or you have relapses (the FSGS comes back). These medicines work on the immune system. Examples include cyclosporine, tacrolimus, mycophenolate mofetil, cyclophosphamide, and rituximab. Talk to your healthcare provider about which medicine is right for you. You can also ask about research studies on FSGS that are trying new treatments.
Your healthcare provider may also recommend:
- diuretics (water pills) and a low-salt diet to help manage swelling
- ACE (angiotensin-converting enzyme) inhibitor or ARB (angiotensin receptor blocker) medicines to manage blood pressure and lower urine protein
- cholesterol-lowering medicine (statins) to manage cholesterol
- blood thinners to prevent blood clots (these are used rarely)
- healthy diet, exercise, and not smoking
What can I expect from treatment?
For most adults with FSGS, treatment starts working in about 2 to 3 months. But it can take up to 4 months to work.
It is very common to relapse and need prednisone again, typically within the first few years. Some people will have many relapses. Your healthcare provider may suggest repeat courses of prednisone or other treatments for this if the prednisone isn’t working or is causing problems. Some of the other medicines may need to be taken for several years.
About half of adults with FSGS already have problems with how their kidneys work (impaired kidney function) by the time they’re diagnosed. Even with treatment, FSGS can scar the kidneys for life and cause kidney failure.
Because FSGS is much less common in children, talk to your healthcare provider about what to expect from your child’s treatment.