Iga Nephropathy

1 What is IgA Nephropathy?

IgA nephropathy (nuh-FROP-uh-thee) is also called Berger's disease as it is named after a French physician, Berger, who described this disease first.

It is a kidney disorder that results due to the accumulation of antibodies called IgA in your kidney tissues.

The most important function of kidneys is to filter toxic wastes, excess water, and electrolytes from your blood, and the glomeruli play a significant role.

As the IgA build up damages the glomeruli, the kidney's ability to clear wastes gets hampered.

Nephropathy or damage to the kidney is indicated by symptoms such as swelling in your feet, the presence of blood and protein in your urine, and high blood pressure.

IgA nephropathy progresses slowly over several years, and in some patients, this loss of kidney function may lead to chronic kidney failure, which needs dialysis or kidney transplant.

There is no cure for IgA nephropathy, but certain medications can slow down its course.

Maintaining your blood pressure in normal limits and reducing your cholesterol levels can slow progression of the disease.

2 Symptoms

IgA nephropathy may not cause any symptoms for many years when the disease is in its early stages.

The disease goes unnoticed for decades and is first identified when routine urine tests reveal the presence of protein and red blood cells in your urine that is not visible without a microscope (microscopic hematuria).

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

  • Cola- or tea-colored urine due to red blood cells in the urine (starts during or after respiratory infection)
  • Repeated episodes of cola- or tea-colored urine, sometimes even visible blood in your urine
  • Pain in the side(s) of your back just below your ribs (flank area)
  • Increased frequency of urination, especially at night
  • Fatigue
  • Nausea
  • Foam in the toilet water from protein in your urine
  • Swelling (edema) in your hands and feet
  • High blood pressure

Consult your doctor if you notice blood in your urine. Urinary bleeding may due to strenuous exercises, certain foods, medications or a urinary tract infection.

Prolonged or repeated urinary bleeding may be a sign of a serious medical problem and should always be investigated for the cause.

Also, seek your doctor's advice if your urine is foamy or if you suddenly develop swelling in your hands and feet.

3 Causes

The exact cause of IgA nephropathy is unknown.

Immunoglobulin A (IgA) is produced by glands situated near your throat and bowel.

It is an antibody that plays an important role in our immune system by attacking invading pathogens and fighting off infections.

In IgA nephropathy, as the antibody travels along the blood circulation and passes through the kidney, it gets settled in the glomeruli leading to an inflammatory reaction (glomerulonephritis), and gradually hampers the filtering ability.

The exact reason for deposition of IgA in the kidneys is not known, but following conditions or factors may be associated with the development of IgA nephropathy:

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

4 Making a Diagnosis

If you have signs and symptoms of IgA nephropathy, you may initially consult your primary care doctor to receive a diagnosis.

Then, you may be referred to a doctor who specializes in the treatment of kidney disorders (nephrologist).

What you can do Getting ready for your appointment:

  • Ask if any pre-appointment restrictions are to be taken, such as fasting for a blood test, when you make the appointment.
  • Make a list of your symptoms, including those that seem unrelated to the reason for your appointment.
  • Make a list of all your regular medications and their doses, including over-the-counter drugs, vitamins, herbal or other supplements or take the original bottles to your appointment.
  • Ask a family member or a friend to accompany you as they can help you remember the information provided during your appointment.

Prepare questions you may want to ask your doctor. Some basic questions include:

  • What tests do I need to undergo to determine if I have IgA nephropathy?
  • What is a kidney biopsy and how is it done?
  • Is this condition a temporary or long lasting problem?
  • What are my chances of developing kidney failure? Is there any method to know?
  • What is the best course of action?
  • How long should I take the medications?
  • What are the alternatives to the primary approach that you have suggested?
  • Is diet and lifestyle changes sufficient to manage this disease?
  • How can I best manage my other health problems together?
  • I have had urinary tract infections in the past. Could these previous infections be a factor?

Your doctor may ask you several questions, which include:

  • When were your symptoms noticed first?
  • Have your symptoms been continuous or occasional?
  • Are your symptoms severe?
  • Does anything seem to improve or worsen your symptoms?

IgA nephropathy is often suspected when you notice bloody urine or if a routine urine analysis reveals protein or blood in your urine. These could be signs of several types of kidney disease.

To accurately identify your problem, further tests may be performed:

Urine test: Blood or protein in the urine may be the initial sign of IgA nephropathy. This may be identified during a routine checkup.

If your doctor suspects that you could have more severe problems with your kidneys, you may need to collect your urine for a 24-hour period for additional kidney function tests to be performed.

Blood tests: If you have kidney disease, such as IgA nephropathy, a blood test may show increased blood creatinine levels.

Iothalamate clearance test: Your doctor may also order an iothalamate clearance test, which involves use of a special contrast agent to track how well your kidneys are filtering wastes.

Kidney imaging: Ultrasound or X-rays also may be used to evaluate the shape or size of your kidneys. A cystoscopy can rule out other causes of bleeding in the urinary tract.

Kidney biopsy: The only test to confirm a diagnosis of IgA nephropathy is a kidney biopsy.

In this procedure, a special biopsy needle is used to take out small pieces of kidney tissue, which is examined under a microscope to determine if there are IgA deposits in the glomeruli.

5 Treatment

Treatment options involve medications that slow down the progression of IgA nephropathy and help manage symptoms such as:

  • high blood pressure,
  • protein in the urine (proteinuria),
  • swelling (edema) in your hands and feet.

There is no definitive cure for IgA nephropathy and no method to know the course of the disease.

A few patients experience complete remission and others lead a normal life with low-grade blood or protein in their urine (hematuria or proteinuria).

Medications used to treat IgA nephropathy include:

High blood pressure medications: Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) lower your blood pressure and also decrease the protein (albumin) in your urine.

Omega-3 fatty acids: These are beneficial fats, available as dietary fish oil supplements. These may reduce inflammation in the glomeruli without causing harmful side effects.

Take your doctor's advice before you start using any supplements.

Immunosuppressive drugs: Corticosteroid medications such as prednisone, and other potent drugs that suppress the immune response (immunosuppressants) may be used to help protect your kidney function.

However, these drugs can cause a range of serious side effects such as high blood pressure or high blood sugar. Therefore, their benefits must be carefully weighed against the risks.

Statin therapy: Cholesterol-lowering medications can slow down the damage to your kidneys.

Mycophenolate mofetil (CellCept): Most recent studies have failed to show a benefit of this medication, but it has been successfully used in some people who have a persistent presence of protein in their urine in spite of treatment with medications that lower blood pressure.

The ultimate goal of treatment is to avoid the need for kidney dialysis or kidney transplantation. In more advanced cases, dialysis or transplant may be necessary

6 Prevention

It is often not possible to prevent IgA nephropathy as its precise cause is unknown.

If you have a family history of IgA nephropathy, it is important to take some steps to maintain your kidney's health by reducing your high blood pressure and cholesterol levels.

7 Lifestyle and Coping

Lifestyle modifications are necessary in order to cope with IgA nephropathy.

In order to keep your kidneys healthy, follow these measures:

Take steps that help reduce your blood pressure: Keeping your blood pressure levels normal helps in slowing down kidney damage caused by IgA nephropathy.

Your doctor may advise healthy changes in your diets such as limiting your intake of salt and other lifestyle changes such as being physically active, losing excess weight, limiting the use of alcohol, and taking your blood pressure medications as ways to maintain your blood pressure under control.

Self-monitoring your blood pressure levels at home: Note each reading and show it to your doctor during your appointments.

Reduce protein intake: Eating fewer amounts of protein and keeping cholesterol levels under control slows the progression of IgA nephropathy and thereby protects your kidneys.

Coping with severe forms of IgA nephropathy can be difficult, but you do not have to do it alone. If you have any questions or need guidance, discuss with a member of your health care team.

You may also consider joining a support group, which provides both empathetic listening and helpful information.

To find out about support groups in your place that deal with kidney disease, you ask your doctor or contact the National Kidney Foundation (NKF) to learn about PEERS Lending Support, a national, telephone-based peer support program from NKF. Call 855-653-7337 to participate.

8 Risks and Complications

There are several risks and complications associated with IgA Nephropathy.

The following risk factors may be involved in the development of this condition:

  • Family history: IgA nephropathy appears to run in families, which indicates that genetic factors contribute to the disease.
  • Your gender: In North America and western Europe, IgA nephropathy is twice more common in men than women.
  • Ethnicity: IgA nephropathy is more common in Caucasians and Asians than it is in blacks.
  • The course of IgA nephropathy is variable. Some people may live with the disease for years with very few problems.

In fact, many cases may go undiagnosed. Other people develop one or more of the following complications:

  • High blood pressure: This causes damage to the kidneys, and strains your heart and rest of the circulatory system.
  • High cholesterol: High levels of cholesterol can enhance your risk of a heart attack.
  • Acute kidney failure: If IgA deposits disrupt the filtering ability of your kidneys, waste products accumulate quickly in your blood.
  • Chronic kidney failure: Long -term IgA nephropathy can lead to gradual loss of filtering capacity in your kidneys.
  • In these cases, permanent dialysis or a kidney transplant is required to sustain life.
  • Nephrotic syndrome: This is a complex of symptoms that result from 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.

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