IgA Nephropathy (Berger’s Disease)

In 1968, French nephrologist Dr. Jean Berger first described immunoglobulin A (IgA) nephropathy. Sometimes referred to as Berger’s disease, IgA nephropathy is a kidney disease that causes the kidneys to become inflamed. At first the disease was believed to be of little threat. But after more and more researchers looked into IgA nephropathy, it turned out that as many as 50 percent of the cases progressed to end stage renal disease (ESRD), or kidney failure, according to the IgA Nephropathy Support Network. After diabetes and high blood pressure, IgA nephropathy is the third leading cause of chronic kidney disease (CKD).

No cure exists for IgA nephropathy, but certain medications can slow its course. Keeping your blood pressure under control and reducing your cholesterol levels also slow disease progression.

SYMPTOMS OF IgA NEPHROPATHY

IgA nephropathy usually doesn’t cause symptoms in the early stages. The disease can go unnoticed for decades and is sometimes first suspected when routine tests reveal protein and red blood cells in your urine that can’t be seen without a microscope (microscopic hematuria).

Signs and symptoms of IgA nephropathy when kidney function is impaired include:

  • Cola- or tea-colored urine (caused by red blood cells in the urine)
  • Repeated episodes of cola- or tea-colored urine, sometimes even visible blood in your urine, usually during or after an upper respiratory or other type of infection
  • Pain in the side(s) of your back below your ribs (flank)
  • Swelling (edema) in your hands and feet
  • High blood pressure
  • Periodic pain in the loins, abdomen, sides or flanks
  • Foam after urination caused by protein in the urine (known as proteinuria)
  • Fatigue
  • Flu and cold-like symptoms
  • High blood pressure
  • Swelling of the hands and feet (edema)
  • Mood swings
  • Becoming more susceptible to allergies
  • Lack of response to cold temperatures (mainly in children)
  • Urinary tract infections (UTIs, mainly in young girls)
  • If you find blood in the urine, talk to your doctor immediately, because this could be a symptom for IgA nephropathy

 

When to see a doctor

Make an appointment with your doctor if you notice blood in your urine. Urinary bleeding may be caused by strenuous exercise, some foods, medications or a urinary tract infection.

But prolonged or repeated bleeding may indicate a serious medical problem and should always be evaluated. Also see your doctor if you develop sudden swelling in your hands and feet.

CAUSES OF IgA NEPHROPATHY

Your kidneys are two bean-shaped, fist-sized organs located at the small of your back, one on each side of your spine. Each kidney contains tiny blood vessels (glomeruli) that filter waste, excess water and other substances from your blood as they pass through your kidneys. The filtered blood re-enters your bloodstream, while the waste material passes into your bladder and out of your body when you urinate.

Immunoglobulin A (IgA) is an antibody that plays a key role in your immune system by attacking invading pathogens and fighting infections. But in IgA nephropathy, this antibody collects in the glomeruli, causing inflammation (glomerulonephritis) and gradually affecting their filtering ability.

Researchers don’t know exactly what causes IgA deposits in the kidneys, but these conditions or factors may be associated with the development of IgA nephropathy:

  • Genes, because IgA nephropathy is more common in some families and in certain ethnic groups
  • Liver diseases, including cirrhosis, a condition in which scar tissue replaces normal tissue within the liver, and chronic hepatitis B and C infections
  • Celiac disease, a digestive condition triggered by eating gluten, a protein found in most grains
  • Dermatitis herpetiformis, an itchy, blistering skin disease that stems from gluten intolerance
  • Infections, including HIV infection and some bacterial infections

Risk factors

Although the exact cause of IgA nephropathy is unknown, these factors may increase your risk of developing this condition:

  • Sex. In North America and western Europe, IgA nephropathy affects at least twice as many men as it does women.
  • Ethnicity. IgA nephropathy is more common in Caucasians and Asians than it is in blacks.
  • Family history. In some cases, IgA nephropathy appears to run in families, indicating that genetic factors may contribute to the disease.

Complications

The course of IgA nephropathy varies from person to person. Some people have the disease for years with few problems. In fact, many cases may go undiagnosed. Other people develop one or more of the following complications:

  • High blood pressure. Damage to your kidneys from IgA deposits can raise your blood pressure, and high blood pressure can cause further damage to your kidneys.
  • High cholesterol. High levels of cholesterol may increase your risk of a heart attack.
  • Acute kidney failure. If your kidneys lose their filtering ability due to IgA deposits, waste products build up quickly in your blood.
  • Chronic kidney disease. IgA nephropathy can cause your kidneys to gradually stop functioning. In such cases, permanent dialysis or a kidney transplant is needed to sustain life.
  • Nephrotic syndrome. This is a group of problems that can be caused by damage to the glomeruli, including high urine protein levels, low blood protein levels, high cholesterol and lipids, and swelling of your eyelids, feet and abdomen.

Prevention

Because the cause of IgA nephropathy isn’t known, it’s not possible to prevent it. But if you have a family history of IgA nephropathy, talk with your doctor to find out what steps you can take to keep your kidneys healthy, such as reducing high blood pressure and keeping your cholesterol at healthy levels.

DIAGNOSING IgA NEPHROPATHY

IgA nephropathy is often detected after you notice blood in your urine or when a routine test shows you have protein or blood in your urine. These could be signs of several types of kidney disease. To identify your problem, these tests may be performed:

  • Urine test: Blood or protein in the urine may be the first sign of IgA nephropathy. This may be discovered as part of a routine checkup. If your doctor suspects that you have problems with your kidneys, you may need to collect your urine for a 24-hour period for additional kidney function tests.
  • Blood tests: If you have kidney disease, such as IgA nephropathy, a blood test may show increased blood levels of the waste product creatinine.
  • Kidney biopsy: The only way for your doctor to confirm a diagnosis of IgA nephropathy is with a kidney biopsy. This procedure involves using a special biopsy needle to extract small pieces of kidney tissue for microscopic examination to determine if there are IgA deposits in the glomeruli.
  • Iothalamate clearance test: Your doctor may also recommend an iothalamate clearance test, which uses a special contrast agent to track how well your kidneys are filtering wastes.

TREATING IgA NEPHROPATHY

There’s no cure for IgA nephropathy and no definitive way of knowing what course the disease will take. Some people experience complete remission and others live normal lives with low-grade blood or protein in their urine (hematuria or proteinuria).

Treatment with a number of medications can slow the progress of the disease and help you manage symptoms such as high blood pressure, protein in the urine (proteinuria), and swelling (edema) in your hands and feet.

Medications used to treat IgA nephropathy include:

  • High blood pressure medications. A common complication of IgA nephropathy is high blood pressure. Taking angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) can lower your blood pressure and reduce the amount of protein (albumin) in your urine.
  • Omega-3 fatty acids. These beneficial fats, available in dietary fish oil supplements, may reduce inflammation in the glomeruli without harmful side effects. Get advice from your doctor before you start using supplements.
  • Immunosuppressants. In some cases, corticosteroid medications, such as prednisone, and other potent drugs that suppress the immune response (immunosuppressants) may be used to help protect kidney function. But these drugs can cause a range of serious side effects, such as high blood pressure or high blood sugar, so their benefits must be carefully weighed against the risks.
  • Statin therapy. Cholesterol-lowering medications may help to slow damage to your kidneys.
  • Mycophenolate mofetil (CellCept). Most studies so far have failed to show a benefit for using this medication, but it has been used successfully in some people who have persistent protein in their urine despite treatment with medications that lower blood pressure.

The ultimate goal is to avoid the need for kidney dialysis or kidney transplantation. But in more advanced cases, dialysis or transplantation may be necessary.

Lifestyle and home remedies

To help keep your kidneys healthier:

  • Take steps to reduce your blood pressure. Keeping your blood pressure levels near normal may help slow kidney damage from IgA nephropathy. Your doctor may recommend healthy changes in your diet — including limiting your salt intake — losing excess weight, being physically active, using alcohol in moderation and remembering to take your blood pressure medications as ways to keep your blood pressure under control.
  • Monitor your blood pressure levels at home. Note each reading and bring this record with you to your doctor’s appointments.
  • Eat less protein. Reducing the amount of protein you eat and taking steps to decrease your cholesterol levels may help slow the progression of IgA nephropathy and protect your kidneys.

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