Glomerulosclerosis, focal

Focal Segmental Glomerulosclerosis (FSGS) Focal Segmental Glomerulosclerosis (FSGS) is a rare kidney disorder characterized by scarring (sclerosis) in the kidney's filtering units, known as glomeruli. This condition can lead to significant kidney damage and is a common cause of nephrotic syndrome in adults and children.

Pathophysiology

FSGS is defined by the presence of sclerosis in some (focal) and not all (segmental) glomeruli. The scarring disrupts the normal filtering process of the kidneys, leading to proteinuria, or the leakage of protein into the urine. Over time, this can result in decreased kidney function and potentially lead to kidney failure.

Causes

The exact cause of FSGS is often unknown, but it can be classified into primary and secondary forms:

• Primary FSGS: The cause is idiopathic, meaning it arises spontaneously or from an obscure or unknown cause.
• Secondary FSGS: This form can result from other conditions such as obesity, hypertension, or infections like HIV. It can also be associated with drug use or genetic mutations.

Symptoms

The symptoms of FSGS can vary but often include:

• Proteinuria
• Edema (swelling), particularly in the legs and around the eyes
• Hypertension (high blood pressure)
• Hyperlipidemia (high levels of fats in the blood)
• Reduced kidney function

Diagnosis

Diagnosis of FSGS typically involves a combination of:

• Urinalysis to detect proteinuria
• Blood tests to assess kidney function
• Kidney biopsy to identify the characteristic scarring in the glomeruli

Treatment

Treatment for FSGS aims to reduce proteinuria and protect kidney function. Options may include:

• Corticosteroids or other immunosuppressive drugs
• ACE inhibitors or ARBs to control blood pressure and reduce proteinuria
• Diuretics to manage edema
• Lifestyle changes, such as dietary modifications and weight management

Prognosis

The prognosis for individuals with FSGS varies. Some may respond well to treatment and maintain stable kidney function, while others may progress to end-stage renal disease (ESRD) requiring dialysis or kidney transplantation.

Research and Future Directions

Ongoing research is focused on understanding the genetic and molecular mechanisms underlying FSGS, which may lead to more targeted therapies in the future.

See Also

• Nephrotic Syndrome
• Chronic Kidney Disease
• Kidney Biopsy

External Links

• [National Kidney Foundation](https://www.kidney.org/)
• [NephCure Kidney International](https://nephcure.org/)

NIH genetic and rare disease info

• NIH info on Glomerulosclerosis, focal

Glomerulosclerosis, focal is a rare disease.