What is FSGS?

Focal segmental glomerulosclerosis (FSGS) is a disease that causes scarring in your glomeruli, making it a glomerular disease. It happens when small parts or sections of these tiny filters scar or harden (sclerosis)

There are three types: Primary FSGS, Secondary FSGS, and Familial FSGS. Primary has no known reasons or obvious causes for happening. In secondary, its onset is due to another disease or condition. This could be from infections, to medication, to drug use, to sickle cell anemia, to obesity, etc. And in familial, it is due to genetics, meaning your biological parents have the genetic mutation for FSGS.

Who does it impact?

FSGS is a rare disease in that only 7 individuals out of a million will get diagnosed with it. It affects both adults and children and it is most common in men >45 years old and black individuals.

Symptoms 

Symptoms include edema in the arms, leg, or face, high cholesterol, below-average albumin (protein in the blood), protein in the urine, sudden weight gain by extra fluids, and hypertension.

Complications include unmanaged high blood pressure, anemia, nausea, vomiting, loss of appetite, and swelling.

To diagnose, a blood test to measure protein, waste, and fat levels is needed. GFR blood tests are used to see how well the kidneys work. Urine tests are needed to measure levels of blood and protein. Genetic testing is needed to see if it was inherited from parents. Kidney biopsies can also be used to diagnose the condition.

Treatment and management depend 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 people with secondary FSGS, treating the underlying condition may stop the kidney damage from progressing.

How to treat and prevent FSGS?

Some further treatments for FSGS in Angiotensin-converting enzyme inhibitors (ACE-Is), where this medication will help lower blood pressure and manage the onset of hypertension by FSGS. Some common side effects of ACE-Is are a dry cough, dizziness, headache, drowsiness, fatigue, and weakness. Some less common side effects include a metallic taste when eating, an upset stomach, and rashes. Some severe side effects include angioedema (swelling of the entire body), jaundice, and allergic reactions. Be careful when taking ACE-Is with NSAIDs (non-steroidal anti-inflammatory drugs), while pregnant or breastfeeding, and medication that affects potassium/sodium levels. Mineralocorticoid receptor blockers (MRBs) are aldosterone receptor antagonists, that work against aldosterone, a hormone that raises blood pressure. MRBs make kidneys move more water and salt into your urine and also prevent you from losing too much potassium, essentially acting as a diuretic. Some side effects include coughs, dizziness, headache, diarrhea, high potassium, and spironolactone.

Changes to your diet and lifestyle can also help slow scarring and the progression of the disease. Limiting how much sodium and protein you eat, exercising daily, quitting smoking if you do smoke, and maintaining a balanced diet are all ways to help.

Prevention of FSGS comes down to maintaining and leading a healthy lifestyle, treating conditions that are known causes of FGS, and seeing your healthcare provider for yearly checkups to keep an eye on your general and kidney health.