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Berger's Disease


Berger's disease is generally believed to be an autoimmune disorder that results in kidney damage and may lead to kidney failure. An autoimmune disorder is a condition in which the person produces antibodies to his or her own tissues.

What is going on in the body?

The job of the kidneys is to make urine by filtering the blood. In Berger's disease, the filtering parts of the kidney, called the glomeruli, become damaged. The damage is due to deposits of proteins called antibodies. Antibodies are normally made by the immune system to fight infections. For some unknown reason, people with Berger's disease have either increased production or reduced clearance of IgA antibodies. The higher level of antibodies clogs up the filtering system of the kidney.

What are the signs and symptoms of the disease?

A person with Berger's disease may have no symptoms at all or may have symptoms that include: ·  blood in the urine, often after a respiratory infection · groin pain, usually at the same time as the blood in the urine ·  high blood pressure · protein in the urine

What are the causes and risks of the disease?

Berger's disease is generally believed to be an autoimmune disorder in which IgA antibodies interfere with normal kidney function. Fifty percent of the people with Berger's disease have a gene known as HLA-DR4. This points to a genetic component to the disease.

New research findings suggest that autoimmune disorders may be triggered by a transfer of cells between the fetus and the mother during pregnancy. The study involved women with scleroderma, an autoimmune disorder involving the skin. These women have more fetal cells in their blood decades after a pregnancy than women who don't have scleroderma. While further research is needed to substantiate these findings, the study does offer an explanation for the much higher incidence of autoimmune disorders in women than in men.

Berger's disease usually occurs in people between the ages of 15 and 35. It is more common in Native Americans than in any other ethnic group. It is rare in black people and more common in Caucasians. Berger's disease is the leading cause of acute kidney disease in young people in the United States, Europe, and Japan. It is more common in Asia and Australia than in the U.S. Males are more often affected than women.

What can be done to prevent the disease?

There are no known ways to prevent Berger's disease.

How is the disease diagnosed?

Diagnosis of Berger's disease begins with a medical history and physical exam. A urine test called a urinalysis will show blood, and often protein, in the urine. This often makes the healthcare provider suspect Berger's disease. A blood test can be done to measure the level of IgA antibodies, which is often higher than normal.

A kidney biopsy is usually needed to confirm the diagnosis. This involves inserting a special needle through the skin of the back and into the kidney. A small piece of kidney is removed with the needle. This piece can then be sent to the lab for examination.

What are the long-term effects of the disease?

 

Some people with Berger's disease, especially children, suffer no long-term effects. In other cases, the disease slowly gets worse over time. A person may develop high blood pressure due to the kidney damage.

Roughly 20% of people with Berger's disease will slowly develop kidney failure, which is the most feared long-term effect. Those with kidney failure need dialysis or a kidney transplant to survive. Dialysis is a procedure to filter the blood. It usually requires the person to be hooked up to a special blood-filtering machine three times a week. This continues for life or until the person gets a kidney transplant.

What are the risks to others?

Berger's disease is not contagious and poses no risks to others.

What are the treatments for the disease?

Treatment of Berger's disease usually focuses on controlling high blood pressure and reducing protein loss in the urine. This is usually done with one of the blood pressure medications called ACE inhibitors. Examples include enalapril and captopril. Fish oil and corticosteroids such as prednisone may help slow down the kidney damage in some cases.

Conditions that aggravate kidney failure, such as infections and obstructions, should be treated promptly. Dietary modifications are important as kidney failure progresses. Individuals should follow the diet prescribed by the healthcare provider, which generally focuses on limiting sodium, protein, and fluid.

A person who develops kidney failure will need dialysis or a kidney transplant.

What are the side effects of the treatments?

ACE inhibitor medications may cause cough, salt imbalances, and kidney problems in some cases. Fish oil capsules can cause burping and a fishy aftertaste. Corticosteroids can cause osteoporosis and an increased risk of infection.

Both dialysis and kidney transplant require surgery. All surgery carries a risk of bleeding, infection, and allergic reaction to anesthesia.

Dialysis has many possible side effects, including infection, salt imbalances, and even death. After a kidney transplant, the person must take powerful medications to prevent rejection. These medications can lead to infections and also may damage the new kidney.

What happens after treatment for the disease?

Treatment does not cure Berger's disease. Affected people, especially children, may go into remission and need no further treatment. Others will have occasional flare-ups of the disease, requiring closer monitoring and treatment. Kidney failure from Berger's disease is usually not reversible. A person with kidney failure usually needs treatment for life.

How is the disease monitored?

The healthcare provider will order regular kidney function tests and check the person's blood pressure periodically. The person can watch for blood in the urine at home. Any new or worsening symptoms should be reported to the healthcare provider.


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