IgA nephropathy

What is IgA nephropathy?

IgA nephropathy (nuh-FROP-uh-thee) is a disease that causes inflammation (swelling) and damage to your kidneys. 

In IgA nephropathy, proteins that are made by your immune system (immunoglobulin A or IgA) get into your kidneys. These proteins build up and form clumps inside the tiny blood vessels in your kidneys that clean your blood (glomeruli). Over time, this buildup causes inflammation and damage to the glomeruli and lowers your kidneys' ability to filter waste and fluid from your blood. 

Kidney damage from IgA nephropathy can be serious and lead to: 

• Chronic kidney disease (CKD)
• Kidney failure (also called end-stage renal disease or ESRD and end-stage kidney disease or ESKD)
• Death

It is one of the most common causes of kidney disease, behind high blood pressure and diabetes. IgA nephropathy is also known as Berger's disease.

What are the signs and symptoms of IgA nephropathy?

Kidney damage from IgA nephropathy can take years to develop. People with IgA nephropathy often do not have any symptoms for 10 years or more, and some people can have IgA nephropathy and not know it.

The first signs of IgA nephropathy are usually blood or protein in your urine (pee). You may notice:

• Urine that is pink or the color of tea or cola, especially after you have had a cold, sore throat or respiratory infection
• Blood in your urine that you can see
• Foamy urine

Doctors sometimes find signs of IgA nephropathy during a routine urine test, when they see blood or protein in your urine under a microscope. 

You may also:

• Feel weak and tired
• Feel like you have a cold or the flu
• Have pain in your lower back or sides
• Have swelling in your hands or feet
• Have high blood pressure 

What causes IgA nephropathy?

Doctors do not know exactly what causes IgA nephropathy. It is not clear why some people have it while others do not, but people with some health conditions have a higher chance of having it, including: 

• Celiac disease: An allergy to gluten that tends to run in families (gluten is a protein found in wheat, barley and rye) 
• HIV (human immunodeficiency virus) infection
• Cirrhosis: A liver disease in which scar tissue replaces normal liver tissue

IgA nephropathy is also more common in:

• Men
• People of Asian or European descent
• Native American people of the Zuni and Navajo tribes

IgA nephropathy can be passed down through your family, and doctors think it is genetic. However, most cases happen in people with no family history of the disease.  

Even though IgA nephropathy is caused by proteins made by your immune system, IgA nephropathy is not considered an autoimmune disease (diseases that cause your body's immune system to attack itself).

How can doctors tell if I have IgA nephropathy?

If doctors find protein in your urine after having your urine tested, they may suspect IgA nephropathy. Because other kidney problems can cause protein in your urine, the only way doctors can be sure you have IgA nephropathy is by performing a kidney biopsy. A kidney biopsy is a surgery where doctors take out a very small piece of tissue from your kidneys to look at it under a microscope.

You can find out you have IgA nephropathy at any age, but 8 out of 10 (80%) people with IgA nephropathy are diagnosed between ages 16 and 35. IgA nephropathy is rare in children.

How do doctors treat IgA nephropathy?

There is no cure for IgA nephropathy. The goal of treatment is to slow the damage to your kidneys and delay or prevent kidney failure. Your doctor will work with you to make a treatment plan. 

Doctors treat IgA nephropathy with medicines that: 

• Lower inflammation (swelling)
• Help control your blood pressure
• Help remove extra fluid from your blood
• Lower your cholesterol 

Your doctor may also recommend that you:

• Make changes in what you eat to lower the amount of protein, fat, sodium and cholesterol you take in
• Take in more or less fluids through drinks and some foods 
• Lose weight

Your doctor will also do regular tests to keep track of your kidney health over time.

New treatments being studied

Tonsillectomy

Some research shows that surgery to remove your tonsils (tonsillectomy) may help slow kidney damage from IgA nephropathy, but more research is needed to be sure. 

LNP023

A new treatment called LNP023 is being tested in clinical trials (research studies to see how well a medicine works and if it is safe), but it has not been approved by the FDA for patients in the United States. In the European Union, LPN023 is available to some patients through a program that allows limited access to a medicine before it is fully approved (orphan drug designation). 

Fish oil and omega-3 fatty acids

Researchers are studying fish oil with omega-3 fatty acids as a treatment for IgA nephropathy. Some studies show that fish oil can slow the damage to your kidneys, while other studies show no benefit. More research is needed to know if this treatment works well for IgA nephropathy, but doctors often tell their patients to eat foods high in omega-3, such as walnuts, canola oil and flaxseeds, and to take fish oil supplements because they have a very low chance of causing side effects. 

Treatment for kidney failure from IgA nephropathy 

If you have kidney failure from IgA nephropathy, you will need to go on dialysis or have a kidney transplant. A transplant is not a cure for any type of kidney disease, but is considered a treatment. After a transplant, it is possible the new kidney can eventually become damaged because of igA nephropathy. About 4 out of 10 (40%) transplanted kidneys in people with IgA nephrology eventually fail.

How do people with IgA nephropathy do?

IgA nephropathy affects different people in different ways. It is not possible to predict how IgA nephropathy will affect your health or how long you will live with it. Some people have little or no effect on their health from IgA nephropathy, while others develop kidney disease or kidney failure.  

You are not alone in your IgAN journey. If you'd like to get help and resources from others on the same path, there are several peer support resources to connect you with other IgAN patients and care partners.

How can I prevent IgA nephropathy?

Because it is not clear what causes IgA nephropathy, it is not clear how to prevent it. However, research has shown that for people with IgA nephropathy, medicines that help control your blood pressure and prevent protein from being lost through your urine can help slow damage to your kidneys.

For more resources and information, visit the IgAN Foundation.

Faces of IgA Nephropathy

𝘝𝘪𝘥𝘦𝘰 𝘤𝘰𝘶𝘳𝘵𝘦𝘴𝘺 𝘰𝘧 𝘵𝘩𝘦 𝘐𝘎𝘈 𝘕𝘦𝘱𝘩𝘳𝘰𝘱𝘢𝘵𝘩𝘺 𝘍𝘰𝘶𝘯𝘥𝘢𝘵𝘪𝘰𝘯