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A.D.A.M. Medical Encyclopedia. Atlanta (GA): A.D.A.M.; 2011.

A.D.A.M. Medical Encyclopedia.

Focal segmental glomerulosclerosis

Segmental glomerulosclerosis; Focal sclerosis with hyalinosis

Last reviewed: June 8, 2011.

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.

Causes, incidence, and risk factors

The cause of focal segmental glomerulosclerosis is usually unknown.

The condition affects both children and adults. Men and boys are affected slightly more often than women and girls, and it also occurs more often in African Americans. Focal segmental glomerulosclerosis causes about 20 - 25% of all cases of nephrotic syndrome.

Known causes include:

Symptoms

  • Foamy urine (from excess protein in the urine)

  • Poor appetite

  • Swelling, called generalized edema, from fluids held in the body

  • Weight gain

Signs and tests

The health care provider will perform a physical examination. This may detect tissue swelling and high blood pressure. Signs of kidney (renal) failure and excess fluid may develop as the condition gets worse.

Tests may include:

Treatment

Some patients will receive high doses of corticosteroids or a drug called cyclosporine to suppress their immune system.

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

See also:

In general, treatments may include:

  • Antibiotics to control infections

  • Anti-inflammatory medicines to reduce the immune response

  • Dialysis

  • Fluid restriction

  • Kidney transplant

  • 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

  • Salt-reduced diet

  • Vitamin D supplements

See also: Kidney disease - diet

Expectations (prognosis)

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

Complications

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

  1. Appel GB. Glomerular disorders and nephrotic syndromes. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 122.
  2. 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.

Review Date: 6/8/2011.

Reviewed by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Herbert Y Lin, MD, PhD, Nephrologist, Massachusetts General Hospital; Associate Professor of Medicine, Harvard Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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What works?

  • Immunosuppressive treatment for focal segmental glomerulosclerosis in adults Immunosuppressive treatment for focal segmental glomerulosclerosis in adults
    Focal and segmental glomerulosclerosis (FSGS) is a rare disease whose cause is unknown and is a condition in which the glomeruli leak protein from the blood into the urine. It is described as focal because only some of the glomeruli become scarred (while others remain normal) and segmental as only part of an individual glomerulus is damaged. Over half of all persons with FSGS will develop chronic kidney failure within 10 years. Thus, immunosuppressive strategies are used to control proteinuria and prevent kidney failure. This systematic review identified four studies (108 participants) investigating immunosuppressive treatments for adults with biopsy‐proven FSGS. Adult patients treated with cyclosporin A in combination with prednisone were more likely to achieve partial remission of nephrotic syndrome compared with prednisone alone, however this result is based on only one small study. No data was available on the progression to kidney failure or death.
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Figures

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Drugs of interest

  • Cyclosporine
    Cyclosporine and cyclosporine (modified) are used with other medications to prevent transplant rejection (attack of the transplanted organ by the immune system of the person who received the organ) in people who have received kidney, liver, and heart transplants. Cyclosporine (modified) is also used alone or with methotrexate (Rheumatrex) to treat the symptoms of rheumatoid arthritis (arthritis caused by swelling of the lining of the joints) in patients whose symptoms were not relieved by methotrexate alone. Cyclosporine (modified) is also used to treat psoriasis (a skin disease in which red, scaly patches form on some areas of the body) in certain patients who have not been helped by other treatments. Cyclosporine and cyclosporine (modified) are in a class of medications called immunosuppressants. They work by decreasing the activity of the immune system.
  • Rituximab Injection
    Rituximab is used alone or with other medications to treat certain types of non-Hodgkin's lymphoma (NHL; a type of cancer that begins in a type of white blood cells that normally fights infection). Rituximab is also used with another medication to treat the symptoms of rheumatoid arthritis (RA; a condition in which the body attacks its own joints, causing pain, swelling, and loss of function) in people who have already been treated with a certain type of medication called a tumor necrosis factor (TNF) inhibitor. Rituximab is in a class of medications called biologic antineoplastic agents. It treats NHL by causing the death of blood cells that have multiplied abnormally. It treats rheumatoid arthritis by causing the death of certain blood cells that may cause the immune system to attack the joints.

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