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Focal segmental glomerulosclerosis

Definition

Focal segmental glomerulosclerosis is scar tissue that forms in parts of the kidney called glomeruli. The glomeruli serve as filters, helping rid the body of unnecessary or harmful substances. Each kidney has thousands of glomeruli. One glomeruli is called a glomerulus.

"Focal" means that some of the glomeruli become scarred, while others remain normal. "Segmental" means that only part of an individual glomerulus is damaged.

Alternative Names

Segmental glomerulosclerosis; Focal sclerosis with hyalinosis

Causes, incidence, and risk factors

The cause of focal segmental glomerulosclerosis is usually unknown. A small number of cases result from reflux nephropathy. The condition affects both children and adults. Men and boys are affected slightly more often than women and girls, and it also occurs more frequently in African-Americans. There are also rare, inherited forms of focal segmental glomerulosclerosis.

Focal segmental glomerulosclerosis causes about 10 - 15% of all cases of nephrotic syndrome.

Symptoms

• Foamy urine
• Poor appetite
• Swelling of the body, called generalized edema, from retained fluids
• Weight gain

Signs and tests

The health care provider will perform and physicla examination. This condition is diagnosed based on edema and elevated blood pressure. Signs of kidney (renal) failure and associated fluid overload may develop as the condition gets worse.

Tests may include:

• Immunofluorescence microscopy test
• Kidney biopsy
• Urinalysis

Treatment

Some patients will receive high doses of corticosteroids or a drug called cyclosporine. It is not clear, however, whether these drugs can prevent the kidneys from eventually failing.

The goal of treatment is to control the symptoms associated with nephrotic syndrome and chronic kidney failure.

See also:

• Chronic kidney failure
• Nephrotic syndrome

In general, treatments may include:

• Antibiotics to control infections
• Dialysis
• Fluid restriction
• Kidney transplantation
• Low fat diet
• Low or moderate protein diet (1 gram of protein per kilogram of body weight per day)
• Medicines to treat high blood pressure, high blood cholesterol, and high triglyceride levels
• Powerful anti-inflammatory medicines to reduce the immune response
• Salt-free diet
• Vitamin D supplementation

See also: Kidney disease - diet

Support Groups

Expectations (prognosis)

More than half of those with focal or segmental glomerulosclerosis develop chronic kidney failure within 10 years.

Complications

• Chronic kidney failure
• End-stage kidney disease
• Infection
• Malnutrition
• Nephrotic syndrome

Calling your health care provider

You should call your doctor if you develop symptoms of this condition, especially if there is fever, pain with urination, or decreased urine output.

Prevention

No prevention is known.

References

Nachman PH, Jennette JC, Falk, RJ. Primary Glomerular Disease. In: Brenner BM, ed. Brenner and Rector's The Kidney. 8th ed. Philadelphia, Pa; Saunders Elsevier; 2007: chap 30.