What is Berger's Disease?

Berger's Disease is a kidney ailment. It's clinical name is IgA Nephropathy (IgA referring to the antibodies; Nephropathy for kidneys). I was diagnosed with it in 1991 following an acute kidney failure.  It's not debilitating, but my condition is monitored through frequent blood tests and urinalysis. 

Because it is considered a relatively "new" condition, there was very little information available in 1991.  It remains a lesser-known and seldom-diagnosed disease.  Hopefully putting this information in my website will help others.  The following is intended for research only.  The websites where it originated are noted, along with links.

  

         
From the National Kidney Foundation

IgA Nephropathy

 In many parts of the world, IgA nephropathy is the most common form of glomerulonephritis - a disease that damages the tiny filtering units of the kidney, called glomeruli. The damage caused by IgA nephropathy results from abnormal deposits of a protein called "IgA" in the glomeruli.

One of the kidney's most important jobs is to filter toxic waste products from the blood, and the glomeruli play a key role in this process. As more glomeruli are damaged by the IgA protein, the kidney progressively loses its ability to clear wastes from the body. In some patients with IgA nephropathy, this loss of kidney function progresses to chronic kidney failure, which requires dialysis treatment or a kidney transplant to maintain life.

IgA Nephropathy is sometimes called "Berger's Disease," because a French physician named Berger was one of the first to describe the disease.

What are the signs and symptoms of IgA nephropathy?
The most common sign is blood in the urine. The amount of blood may be so small that it is only visible with the aid of a microscope. Another common sign is swelling of the feet.

As loss of kidney function progresses, symptoms may include pain in the back below the ribs, increased need to urinate (especially at night), fatigue, nausea, swelling of hands and feet, and high blood pressure.

What causes IgA nephropathy?
The causes of IgA nephropathy are not known exactly. The disease seems to cluster in certain families and in certain areas of the world. In addition, it is rare in blacks. These facts suggest that genetic influences may play a role in the development of the disease.

How is IgA nephropathy diagnosed?
The presence of blood and/or protein in the urine may suggest a diagnosis of IgA nephropathy. However, to confirm this diagnosis, it is necessary to do a biopsy. The doctor removes a small piece of tissue from the kidney (biopsy) and examines it under a microscope for the presence of the characteristic IgA deposits in the glomeruli.

How is IgA nephropathy treated?
Efforts to slow the progression of kidney damage may include limiting the amount of protein in the diet and careful control of high blood pressure if present. In addition, a recent study suggested that taking fish oil supplements may also help to slow the loss of kidney function. For patients who develop progressive kidney failure, treatment may consist of dialysis or a kidney transplant. The success rate of transplants is good in these patients. Even though the IgA deposits reappear in the transplanted kidney in about half the patients within one year after the operation, the signs and symptoms of the disease remain mild. Loss of a transplanted kidney to recurrent IgA nephropathy is uncommon. The milder form of the disease seen after transplantation may be due to the use of antirejection drugs such as cyclosporine.

What is the outlook for patients with IgA nephropathy?
About 20-40 percent of the patients develop end stage kidney failure about 20 years after the disease becomes apparent. Those patients who have an increased level of creatinine in their blood at the time of their diagnosis are more likely to develop chronic kidney failure. It is harder to predict which of the patients who have normal levels of creatinine at the time of diagnosis will develop kidney failure. In general, a poor prognosis is expected for those patients who have high blood pressure, a loss of more than 2 grams of protein a day in their urine, and a significant amount of damage present in their biopsy specimen.

What research is being done?
Several centers in the United States and other countries are studying IgA n nephropathy. Researchers are investigating the chemical composition of the IgA protein, the rate of production of this protein, possible genetic influences and analysis of the long-term clinical outcome. Cooperative treatment trials on the effectiveness of the drug prednisone are continuing in the United States. Researchers recently completed a study of the effectiveness of fish oil supplements in slowing of the disease. They reported that fish oil supplements significantly slowed loss of kidney function in patients with IgA nephropathy.

 

From the IgAN Foundation

IgA Nephropathy or Berger's ("burrjays") disease is the most common non-diabetic kidney disease. It results when IgA a normal component of the blood collects in the kidney as damaging deposits. These deposits are an immune system defect, hence IgAN is considered an autoimmune disease. Just why these deposits form is not known though a variety of factors such as family genetics and coincident infections seem to play important roles.

 

Available evidence suggests that IgA nephropathy occurs from either increased production or reduced clearance of the immune protein IgA and associated antigen complexes that are ultimately deposited within the kidney. 

 

Many sources categorize IgAN as a rare disease with some sources claiming it afflicts 1:100,000. It seems that this estimated level of incidence for IgAN is not accurate as a large proportion of patients who present with IgAN symptoms have mild disease which is not diagnosed via the accepted biopsy diagnosis. One published study showed 94 out of 100,000 military inductees were diagnosed with IgAN. Other published research of random autopsy kidney biopsies suggests IgAN may be vastly more common and may affect up to 2-4% of the human population at large. Certainly there is a dramatic variance in the prevalence of diagnosed IgAN. In Japan and France where testing for the condition is part of regular preventative medical care the disease incidence is twice that found in the USA where testing for IgAN is rarely performed as preventative medicine.

 

Most people probably never realize they have the disease or at least do not realize it until a late stage. Amongst those diagnosed as having IgAN as many as 20%- 30% will suffer eventual kidney failure within 10-20 years. They will require life saving dialysis and/or a kidney transplant.

 

A Few Warning Signs of Kidney Disease

  • Tea colored urine - (hematuria - blood in the urine)

  • Very foamy urine - (proteinuria - protein in the urine)

  • Puffiness around the eyes, hands, or feet - (edema - fluid retention)

  • High blood pressure

  • Pain in the small of the back just below the ribs not aggravated by motion.

  • Frequent urge to urinate especially at night and or reduced amount of urine produced.

 There are no "widely accepted" western medical treatments for IgAN save in the latest stages of the disease. There is however growing evidence that a number of therapies can be effective in delaying the deterioration of kidney function for many years. Most nephrologists with an active awareness of IgAN prescribe ACE inhibitors and fish oil at a minimum. In some cases powerful steroid treatment is utilized. For about half of those with IgAN tonsillectomy, which treats part of the underlying immune disorder, is effective. There are additional new treatments that show the promise of being a start on finding a cure for the disease. 

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