IgA Nephropathy

What is IgA nephropathy?

IgA nephropathy (nuh-FROP-uh-thee) is a type of kidney disease. In people with IgA nephropathy, antibodies build up in their kidneys and cause inflammation. The inflammation damages their glomeruli (glo-MARE-yoo-lye). Glomeruli are tiny blood vessels that perform the first step in filtering waste products out of your blood, which eventually leave your body in your urine (pee). Glomerular diseases cause your kidneys to leak blood (hematuria) and protein (proteinuria) into your pee.

Other names for IgA nephropathy include:

• Immunoglobulin A nephropathy
• IgAN
• IgAN disease

Another common name for IgA nephropathy is Berger’s disease. Two French doctors, Jean Berger and Nicole Hinglais, discovered IgA nephropathy in the late 1960s.

What does IgA nephropathy mean?

IgA nephropathy is a big term. But what does it mean?

• Immunoglobulin A (IgA). IgA is an antibody. It’s a protein your immune system makes to protect you from foreign invaders, such as bacteria and viruses.
• Nephropathy. Nephropathy is a medical term that describes any damage, disease or other problem that affects your kidneys.

Is IgA nephropathy life-threatening?

Without treatment, IgA nephropathy may gradually get worse and cause kidney failure (renal failure). Kidney failure is fatal without treatment.

How common is IgA nephropathy?

IgA nephropathy is a common kidney disease that can happen to people at any age.

In the United States, about 1 out of every 10 kidney biopsies show IgA nephropathy. In East Asian countries, IgA nephropathy appears in about 4 out of every 10 kidney biopsies. In European countries, it appears in between 2 and 3 out of every 10 kidney biopsies.

What are the main symptoms of IgA nephropathy?

The most common IgA nephropathy symptoms include:

• Visible blood in your pee
• Flank pain
• Swelling (edema), especially in your legs, ankles, abdominal area or face
• Foamy urine

You may not have any IgA nephropathy symptoms at first. It can be years or even decades before signs appear.

What causes IgA nephropathy?

Medical experts believe IgA nephropathy is an autoimmune disease. That means your immune system attacks your kidneys.

People with IgA nephropathy have a higher level of IgA with less galactose (guh-LAK-tohs) than is typical. Galactose is a type of sugar. Your immune system considers these low-galactose IgA to be foreign invaders. As a result:

• Other antibodies in your body attach to the low-galactose IgA
• The IgA forms masses or clusters (immune complexes)
• The immune complexes get stuck in your glomeruli
• Inflammation and damage result in kidney disease

IgA immune complexes circulate in your body more when you have a cold or upper respiratory infection. Some of these complexes end up in your kidneys. As a result, some people have symptoms of IgA nephropathy after a cold.

Is IgA nephropathy genetic?

For some people, there’s a genetic family history of IgA nephropathy. Medical experts have discovered some genetic markers. That means there may be a link between a genetic variation and IgA nephropathy.

Who does IgA nephropathy affect?

Risk factors for IgA nephropathy include a genetic family history of:

• IgA nephropathy
• IgA vasculitis (Henoch-Schönlein purpura)

Although IgA can affect anyone, you may be more likely to have it if you’re:

• Male.
• In your late teens to late 30s
• Asian or European

In the U.S., males are twice as likely to have IgA nephropathy as females.

When does IgA nephropathy start?

IgA nephropathy can affect anyone at any age. But the first signs of the disease usually occur in a person’s late teens to late 30s.

What are the complications of IgA nephropathy?

IgA nephropathy complications may include:

• High blood pressure (hypertension)
• Sudden loss of kidney function (acute kidney failure)
• Slow loss of kidney function over time (chronic kidney failure)
• Nephrotic syndrome
• Cardiovascular system problems

How is IgA nephropathy diagnosed?

Your healthcare provider will ask about your symptoms and medical and family history. You may have:

• Physical exam, including checking your blood pressure and looking for signs of swelling
• Urinalysis to check for blood in your urine
• Blood tests to check levels of cholesterol, protein and waste in your blood
• Estimated glomerular filtration rate (eGFR) blood test to check how much blood your kidneys are filtering every minute
• Urine protein test to check for protein and/or albumin in your urine
• Twenty-four-hour urine studies to check how much protein or albumin is being lost in your urine during that time period

Will I need a kidney biopsy?

If you need further testing, your healthcare provider may order a kidney biopsy. This procedure can confirm a diagnosis of IgA nephropathy.

Your healthcare provider uses a needle to remove a small piece of kidney tissue. A pathologist examines the tissue to look for IgA deposits in the glomeruli and see the extent of the damage.

How is IgA nephropathy treated?

Treatment can slow the progress of the disease and prevent ESRD. If you have IgA nephropathy, your healthcare provider will probably refer you to a nephrologist, a kidney disease specialist.

Treatment for IgA nephropathy includes medication to:

• Regulate blood pressure with angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs), or other medicines
• Remove extra fluid with a diuretic
• Control your immune system to lower kidney inflammation with prescribed steroids such as prednisone or cyclophosphamide, a different medicine
• Lower your cholesterol levels with medications such as statins

Can a tonsillectomy help people with IgA nephropathy?

Some research shows that a tonsillectomy (tonsil removal) can benefit a small portion of people who have IgA nephropathy. Talk to your healthcare provider to see if this may be an option for you.

How can I prevent IgA nephropathy?

There isn’t a known way to prevent IgA nephropathy. If IgA nephropathy runs in your family, talk to your healthcare provider. They can recommend steps to stay healthy, such as keeping blood pressure and cholesterol well managed.

Does my diet affect whether I will get IgA nephropathy?

Researchers have not found a link between nutrition or eating habits and preventing IgA nephropathy.

What can I expect if I have IgA nephropathy?

It depends. Sometimes, IgA nephropathy gets better with conservative treatment. In rare cases, IgA nephropathy progresses rapidly. Kidney failure can develop within a few years.

With the right treatment, many people can keep their kidneys as healthy as possible and slow the disease’s progression.

About 1 in 4 adults with IgA nephropathy eventually develop kidney failure. About 1 in every 10 to 20 children with IgA nephropathy develop kidney failure.

Will I need a kidney transplant?

It depends. Everyone’s disease progression is different. Some people respond well to treatment and can live with the condition for a long time.

If IgA nephropathy progresses to kidney failure, you may need to consider dialysis or a kidney transplant. Your care team will discuss your treatment options with you.

How long does IgA nephropathy last?

IgA nephropathy is typically a lifelong, progressive kidney disease. That means it usually gets worse as time goes on. But medications can help slow the condition’s progression.

What is the life expectancy of someone with IgA nephropathy?

A 2017 study of adults with IgA nephropathy in the southeastern U.S. found that life expectancy is reduced by a little over 10 years. The average age of death was just under 66 years, while the expected age of death if you don’t have kidney disease was just under 76 years.

A 2019 study in Sweden found that life expectancy is reduced by about 6 years.

Can I live a normal life with IgA nephropathy?

With proper treatment, many people with IgA nephropathy can live long, fulfilling lives. They can continue working, going to school and participating in activities they love.

What foods should you avoid with IgA nephropathy?

Some healthcare providers may recommend diet restrictions if you have IgA nephropathy. This may include cutting back on:

• Salt
• Protein

A provider may also advise you to avoid foods high in saturated fat and cholesterol if you have high cholesterol.

Why should I eat less protein if I have IgA nephropathy?

Proteins break down into products that your kidneys need to filter. Eating too much protein may overwork your kidneys and cause them to decline faster. However, if you don’t eat enough protein, you may develop malnutrition. A healthcare provider may recommend that you see a renal (kidney) dietitian to develop kidney-friendly eating patterns.

If you’re supposed to cut back on how much protein you eat, you may need regular blood tests to check your protein levels.

Do I need omega-3 supplements if I have IgA nephropathy?

If you have IgA nephropathy, some healthcare providers may recommend fish oil supplements that contain omega-3 fatty acids. They may lower blood pressure and slow the progress of the disease.

It’s important to talk to a healthcare provider before you start taking any supplements, including fish oil.

When should I see a healthcare provider?

It’s a good idea to schedule regular wellness checkups with a healthcare provider. Early detection can help prevent IgA nephropathy from developing into kidney failure. You should also work with a provider to help manage any other conditions that can affect your kidneys, including high blood pressure and diabetes.

When should I go to the ER?

Go to the nearest emergency room if you have IgA nephropathy and develop symptoms of kidney failure, including:

• Feeling more tired than usual (fatigue)
• Peeing more or less than expected
• Loss of appetite
• Difficulty sleeping or focusing

What questions should I ask a healthcare provider?

You may wish to ask:

• How will you diagnose IgA nephropathy?
• What treatment do you recommend?
• What are the side effects of your recommended treatment?
• Will a tonsillectomy help?
• Should I see a renal dietitian?
• Should I take fish oil supplements?
• How often do you recommend blood and urine tests?
• Do I need a kidney transplant?
• Are there any clinical trials in which I may be a good candidate?
• Can you recommend a support group for people with IgA nephropathy?