IgA Nephropathy

Overview

What is IgA nephropathy?

IgA nephropathy is a type of kidney disease. The name stands for immunoglobulin A nephropathy (nuh-FROP-uh-thee). IgA is an antibody, a protein your immune system makes to protect you from bacteria and viruses.

In people with IgA nephropathy, IgA builds up in their kidneys and causes inflammation. This inflammation damages kidney tissues.

IgA nephropathy is also called Berger’s disease. Two French doctors, Jean Berger and Nicole Hinglais, discovered the condition.

What’s the difference between IgA nephropathy and selective IgA deficiency?

Both conditions have to do with the protein IgA. People with selective IgA deficiency either don’t have enough IgA or have low levels of it.

How do my kidneys work?

The kidneys are part of your urinary system. Here’s how they typically work:

  • Kidneys: These bean-shaped organs are on each side of your spine. They filter your blood, removing waste and extra water. The waste and water leave your body as urine. The kidneys then reabsorb the water and chemicals that your body needs.
  • Nephrons: The tiny filtering units in each kidney are called nephrons. Each kidney has about a million nephrons.
  • Glomerulus and tubule: Each nephron contains these structures. The glomeruli (plural of glomerulus) are tiny blood vessels that filter blood. The tubules return what you need to your blood and remove waste.

What is the connection between IgA nephropathy and ESRD?

IgA nephropathy attacks the glomeruli. This type of glomerular disease occurs when IgA deposits build up and damage the glomeruli. The damage causes your kidneys to leak blood (hematuria) and protein (proteinuria) into your urine.

Eventually, the nephrons may scar, causing kidney disease. As the scarring progresses, you may develop end-stage kidney (renal) disease (ESRD). This process can happen quickly over the course of months or can take as long as 20 years after your initial diagnosis.

If you develop ESRD, your kidneys can’t work well enough to keep you healthy. You may need:

  • Dialysis, a machine that helps filter your blood.
  • Kidney transplant, surgery to remove your damaged kidney and replace it with a healthy donor kidney.

Who is at risk for IgA nephropathy?

Risk factors for IgA nephropathy include:

How common is IgA nephropathy?

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

Usually, the first signs of the disease occur in a person’s late teens to late 30s. In the United States, men are twice as likely as women to have IgA nephropathy.

Symptoms and Causes

What causes IgA nephropathy?

Researchers believe IgA nephropathy is an autoimmune kidney disease. Your body’s immune system attacks your kidneys.

People with IgA nephropathy have a higher level of IgA with less galactose than is typical. Galactose is a type of sugar. Your body considers these low-galactose IgA “foreign.” As a result:

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

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

Is IgA nephropathy genetic?

For some people, this condition runs in families. Researchers have discovered some genetic markers, meaning that a genetic mutation (change) may cause IgA nephropathy.

What are the symptoms of IgA nephropathy?

At first, you may have no symptoms. It can be years or even decades before signs appear.

The most common symptoms are:

  • Visible blood in your urine (hematuria).
  • Flank pain (in the sides of your back).
  • Ankle swelling (edema).
  • High blood pressure (hypertension).
  • Proteinuria (having too much protein in your urine), which can cause edema (swelling) and foamy urine.

What are the complications of IgA nephropathy?

Complications include:

  • High blood pressure.
  • Acute kidney failure, suddenly losing kidney function.
  • Chronic kidney failure, slowly losing kidney function over time.
  • Nephrotic syndrome, several symptoms together that indicate kidney damage, including high protein in your urine, swelling and high cholesterol.
  • Heart or cardiovascular problems.
  • IgA vasculitis.

Diagnosis and Tests

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 can be done 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.

Management and Treatment

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:

  • Control 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.

Prevention

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 under control.

Does my diet affect whether I will get IgA nephropathy?

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

Outlook / Prognosis

What’s the outlook for people with IgA nephropathy?

Sometimes, IgA nephropathy gets better on its own. In rare cases, IgA nephropathy progresses rapidly. Kidney failure can result within a few years.

There isn’t a cure for this condition. With the right treatment, people can keep their kidneys as healthy as possible and slow the disease’s progression.

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

Will I need a kidney transplant?

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

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

Frequently Asked Questions

When should I see a healthcare provider for IgA nephropathy?

See your healthcare provider if you notice:

How can I take care of myself if I have IgA nephropathy?

If you have kidney disease such as IgA nephropathy, it’s important to:

  • Limit sodium (salt) in foods to help lower blood pressure and reduce swelling.
  • Decrease how much liquid you drink, another way to lower blood pressure and reduce swelling.
  • Eat foods low in saturated fat and cholesterol to reduce levels of fat in your blood.
  • Lifestyle modification such as quitting smoking, diet and exercise, if needed, can also be beneficial.

Should I eat less protein if I have IgA nephropathy?

Some healthcare providers recommend eating less protein if you have any type of kidney disease. That’s because 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. But if you don’t eat enough protein, you may end up with malnutrition, when your body doesn’t get the nutrients it needs. A nutritionist can help you find a balanced diet for your needs. If you’re on a low-protein diet, you may need regular blood tests to check your protein levels.

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

Some healthcare providers recommend fish oil supplements that contain omega-3 fatty acids. Research suggests this approach may lower blood pressure and slow the progress of the disease. Speak to your healthcare provider before you start taking any supplements.

What else should I ask my healthcare provider?

If you have IgA nephropathy, ask your healthcare provider:

  • What medications do you recommend?
  • Should I eat a special diet?
  • Will a tonsillectomy help?
  • Should I take fish oil supplements?
  • How often should I have blood and urine tests?
  • Do I need a kidney transplant?

A note from Cleveland Clinic

IgA nephropathy is a kidney disease caused by a buildup of the antibody IgA in your kidneys. The resulting inflammation damages your kidneys over time. Treatment aims to slow down the disease. For some, the condition may continue progressing despite treatments. But if this happens, dialysis or a kidney transplant can offer relief.

Last reviewed by a Cleveland Clinic medical professional on 12/13/2021.

References

  • American Kidney Fund. IgA Nephropathy. (https://www.kidneyfund.org/kidney-disease/other-kidney-conditions/rare-diseases/iga-nephropathy/) Accessed 12/13/2021.
  • National Institute of Diabetes and Digestive and Kidney Diseases. IgA Nephropathy. (https://www.niddk.nih.gov/health-information/kidney-disease/iga-nephropathy) Accessed 12/13/2021.
  • National Institute of Diabetes and Digestive and Kidney Diseases. Your Kidneys & How They Work. (https://www.niddk.nih.gov/health-information/kidney-disease/kidneys-how-they-work) Accessed 12/13/2021.
  • National Kidney Foundation. IgA Nephropathy. (https://www.kidney.org/atoz/content/iganeph) Accessed 12/13/2021.
  • National Organization for Rare Disorders. IgA Nephropathy. (https://rarediseases.org/rare-diseases/iga-nephropathy/) Accessed 12/13/2021.

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