IgA Nephropathy (IgAN): Symptoms, Causes, Diagnosis, Treatment, and Prevention

What Is IgA Nephropathy? (IgAN)

What Is IgA Nephropathy? (IgAN)
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IgA nephropathy (IgAN, or Berger’s disease) is an autoimmune disease that causes immunoglobulin A (IgA) antibodies to build up in the kidneys. Antibodies are proteins that the immune system makes to protect the body against bacteria and viruses. But a buildup of IgA antibodies damages the glomeruli, or vessels in the kidneys that filter blood, causing them to leak blood and protein into the urine.

This condition progresses differently in each person who has it. Some people with IgAN live for many years without any problems. Others develop complications like high blood pressure, chronic kidney disease, or kidney failure.

IgAN has no cure, but treatment aims to slow the progression of kidney disease and improve quality of life by managing symptoms.

Types of IgA Nephropathy (IgAN)

Doctors divide IgAN into three types, based on the cause and where in the kidneys the antibodies collect.

  • Primary IgA Nephropathy This is the most common type. It starts in the kidneys, and no other condition causes it. In primary IgAN, the body produces too much galactose-deficient IgA1 (Gd-IgA1), a type of IgA. In response, the immune system produces antibodies that attach to Gd-IgA1. When the attached antibodies and Gd-IgA1 deposit in the kidneys, the immune system releases chemicals that inflame and irritate the kidneys' filters.

  • Secondary IgA Nephropathy This type starts when a condition, such as liver disease or another autoimmune disease, prevents the body from clearing IgA. People with this type have symptoms of both IgAN and the underlying condition.

  • IGA-Associated Nephropathy In this group of diseases, IgAN affects the kidneys and other organs. One common form is IgA vasculitis, formerly known as Henoch-Schönlein purpura. IgA builds up in small blood vessels in the kidneys and other parts of the body. People with IgA vasculitis sometimes have damage to their gastrointestinal tract, joints, and kidneys.

Signs and Symptoms of IgA Nephropathy (IgAN)

Symptoms of IgA nephropathy (IgAN) can take years to become noticeable.

The main symptom is blood in the urine, which looks brown, red, or cola-colored. This blood leaks into the urine from damaged blood vessels in the kidneys.

Other common symptoms and signs include the following:

  • Foamy urine (a sign of too much protein in the urine)
  • Swelling in the face, belly, legs, and ankles
  • Pain on the sides of the lower back
  • Rash
  • High blood pressure

  • Tiredness

  • Weakness

Graphic from Everyday Health titled 'IgA Nephropathy Symptoms'. A human silhouette highlights the symptom callouts for fatigue, swelling, weakness, blood in the urine, high blood pressure, lower back pain, foamy urine, and a rash.

Causes and Risk Factors of IgA Nephropathy (IgAN)

Primary IgA nephropathy (IgAN) occurs when an abnormal immune response causes immunoglobulin A (IgA) to deposit in the glomeruli. The condition often starts after a cold or other respiratory infection. As the immune system fights off the virus or bacteria that caused the infection, it makes too much IgA. The extra IgA travels through the blood and gets stuck in the kidneys' filters, causing inflammation and damage.

IgAN isn't an infection. Instead, it results from a problem with the immune system. In fewer than 10 percent of people with primary IgAN, genetic changes cause the immune problem, but in the remaining 90 percent, no obvious cause is apparent.

Secondary IgAN occurs when another condition causes excessive IgA to build up in the blood. Diseases linked to this form of IgAN include:

The following factors may increase your risk for IgAN:

  • A family history of IgAN or IgA vasculitis

  • Male sex; IgA nephropathy affects twice as many males as females in Europe and North America

  • A history of celiac disease, IBD, hepatitis, cirrhosis, or HIV

  • Age between 10 and 40

  • Asian, Pacific Islander, or white race or ethnicity 

How Is IgA Nephropathy (IgAN) Diagnosed?

The diagnosis starts with a physical exam. A kidney specialist (nephrologist) will ask about symptoms like blood in the urine, swelling, and foamy urine. They'll also take a blood pressure reading, since high blood pressure is a common sign of IgAN.

These lab tests check for signs of kidney damage and kidney function.

  • Urinalysis helps doctors identify blood and protein in the urine.
  • Urine tests detect protein and the waste product creatinine in the urine.
  • Blood tests check creatinine levels and measure the estimated glomerular filtration rate (eGFR), which shows how well your kidneys are filtering blood.
A kidney biopsy confirms the diagnosis. For this test, the doctor removes a small piece of the kidney. A lab examines the sample using a special magnifying glass or a technique called immunofluorescence to determine whether the kidney tissue contains IgA deposits.

Treatment and Medication Options for IgA Nephropathy (IgAN)

There's no cure for IgAN. Treatments reduce protein in the urine, slow kidney damage, relieve symptoms, and prevent complications.

 A doctor's recommendations will depend on the extent of your kidney damage and what other health conditions you have.

Medication Options

These are some of the medicines used to treat IgAN.

  • Blood Pressure Medicines Angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs) are usually the first treatments doctors prescribe. These medicines lower blood pressure, reduce urinary protein levels, and slow the progression of IgAN.

  • Sodium-Glucose Cotransporter 2 (SGLT2) Inhibitors This class of medications was developed for diabetes, but they also treat IgAN. SGLT2 inhibitors reduce protein in the urine and slow the disease.

  • Endothelin Receptor [Type A] Antagonists (ERAs) This is a new type of medicine for IgAN. Atrasentan (Vanrafia) and sparsentan (Filspari) widen blood vessels in the kidneys. They reduce the amount of protein lost in the urine and preserve kidney function.

  • Corticosteroids (Steroids) These calm the immune system so it produces less IgA and reduce the amount of protein in the urine. Taking a steroid medicine for six to nine months can slow disease progression. Doctors don’t recommend staying on the medicine for longer because of serious side effects like high blood sugar, infections, and weak bones. The newer targeted-release steroid budesonide (Tarpeyo) has fewer side effects.

  • Complement Factor B Inhibitor This type of drug targets a specific part of the immune system to reduce protein levels in the urine and slow disease progression. Iptacopan (Fabhalta) is the only drug approved in this class.

  • Monoclonal Antibody A four-weekly injected medication called sibeprenlimab-szsl (Voyxact) is one of the more recently approved treatments for IgAN.

     Sibeprenlimab-szsl targets and blocks a protein called a proliferation-inducing ligand (APRIL) that normally drives the production of IgA antibodies in certain immune cells. This reduces the amount of IgA being produced, which, in turn, reduces kidney inflammation and the amount of protein in urine.

Surgery

Surgery is not a standard treatment for IgA nephropathy, but tonsillectomy and kidney transplants may occur for certain people.

Tonsillectomy The tonsils produce some of the body's IgA, and IgA levels often decrease after tonsil removal. Some studies, most of which took place in Asia, found that tonsillectomy slowed the progression of IgA nephropathy. Because more rigorously designed studies haven’t confirmed these findings, doctors don’t recommend tonsillectomy for most people with IgAN.

 But this surgery may be helpful alongside steroids for children with severe IgAN.

Kidney Transplant IgA nephropathy is a progressive disease. Up to half of people with this condition develop kidney failure within 10 years after diagnosis.

 A kidney transplant is an option, but the disease can come back in a transplanted kidney. One study found that nearly 1 in 4 people had a recurrence of IgAN within 15 years after their kidney transplant.

Clinical Trials

Researchers are studying new treatments for IgAN in clinical trials. They're investigating different combinations of medicines and learning how certain drugs affect specific groups of people. Joining a study of an investigational drug could give you a chance to try a new therapy before it's available to the public.

Lifestyle Changes for IgA Nephropathy (IgAN)

Staying generally healthy will also protect your kidneys, lower your blood pressure, and support the management of other medical conditions.

 Your doctor may recommend the following lifestyle changes to manage IgAN and prevent it from getting worse.

Make Changes to Your Diet

Try to follow a balanced diet. Your doctor or a registered dietitian can help you choose a nutritious mix of foods. A few dietary changes are important for IgAN.

  • Limit sodium to 2,000 milligrams (about 1 teaspoon) or less per day. Salt raises blood pressure and increases the amount of protein released in urine.

  • Reduce protein in your diet. You need some protein to keep your body functioning optimally, but too much can be hard on damaged kidneys.

  • Avoid alcohol or limit drinking. Alcohol can raise your blood pressure. It also interacts with some blood pressure medicines in ways that can cause your pressure to rise too high or drop too low.

Avoid Tobacco Smoke

Smoking is generally harmful to your body, with links to cancer, heart disease, and other conditions. Tobacco smoke can speed up IgAN kidney damage. This, in turn, increases your risk for kidney failure. If you've had trouble quitting, ask your doctor about medications and other tools to help you stop smoking.

Take Care With Medicines That Harm the Kidneys

Some medicines protect the kidneys, while others can cause further damage. These are a few drugs to limit or avoid:

  • Blood Thinners apixaban (Eliquis), dabigatran (Pradaxa), rivaroxaban (Xarelto), and warfarin (Coumadin)
  • Diabetes Drugs glyburide (Diabeta, Glynase), glimepiride (Amarl), insulin, and metformin (Glucophage)
  • Heartburn Medicines proton pump inhibitors (PPIs) like esomeprazole (Nexium), lansoprazole (Prevacid), and omeprazole (Prilosec) and H2 blockers like cimetidine (Tagamet) and famotidine (Pepcid)
  • Nonsteroidal Anti-Inflammatory Drugs (NSAIDs) ibuprofen (Advil, Motrin), naproxen sodium (Aleve), and aspirin
  • Other Medications certain antibiotics, antifungal drugs, and antiviral drugs
Many medications remain safe for people with long-term kidney problems at a lower dose or with less frequent use. Speak to your doctor about the right medication and dosage for your eGFR and medical needs.

IgA Nephropathy (IgAN) Prognosis

IgAN is a lifelong, progressive disease. Eventually, it can lead to kidney failure, which is life-threatening.

Up to half of people with this condition develop kidney failure within 10 years after they're diagnosed.

Every person with IgAN experiences a different disease progression. Some people respond well to treatment and notice that their symptoms improve. Others progress to kidney failure in a few years.

 High levels of protein in your urine increases the risk of kidney failure within 10 years, according to research.

Once you have kidney failure, you'll need dialysis or a kidney transplant.

IgA nephropathy isn't curable, but treatments can extend your lifespan and improve your quality of life. The right treatment plan will help protect your kidneys and slow the disease.

Complications of IgA Nephropathy (IgAN)

IgA nephropathy damages the kidneys and causes blood and protein to leak into the urine. Over time, it can lead to complications like these.

  • Chronic Kidney Disease (CKD) The risk is higher in people who have another condition that harms the kidneys, such as diabetes or high blood pressure.

  • Nephrotic Syndrome Damage to the kidneys' filters causes a group of problems, such as high protein in the urine, high cholesterol, and swelling.

  • High Blood Pressure Damaged kidneys don't control blood pressure as effectively as healthy kidneys.

  • Cardiovascular Disease People with IgAN have high blood pressure and high cholesterol, which increase their risk for a heart attack, stroke, and heart failure.

  • Kidney Failure These organs can become so damaged that they can no longer filter the blood.

Support for People With IgA Nephropathy (IgAN)

IgA Nephropathy Foundation

This is the only organization that's solely dedicated to helping people with IgAN and their caregivers. The IgA Nephropathy Foundation funds research to find a cure for this condition. It also offers information and a variety of support services.

National Kidney Foundation

The National Kidney Foundation is working to stop preventable types of kidney disease. People with IgAN and their caregivers can find information about treatments, resources, and clinical trials on the organization's website.

FAQ

1How do I treat IgA nephropathy?
Blood pressure medications, sodium-glucose cotransporter 2 (SGLT2) inhibitors, endothelin receptor [type A] antagonists (ERAs), steroids, and complement factor B inhibitors can lower blood pressure and reduce protein in the urine.

IgA nephropathy doesn't shorten lifespan itself. But it can lead to kidney failure, which is life-threatening. Treatment can prevent or delay kidney failure and protect life expectancy.

This condition can be very serious. It leads to kidney failure in up to half of people with this condition within 10 years of diagnosis.

People with IgAN should eat a low-sodium diet. Salt contributes to high blood pressure and kidney damage. Try to eat nutrient-rich foods like vegetables, fruits, beans, eggs, and fish. Doctors sometimes recommend limiting protein.

Resources We Trust

EDITORIAL SOURCES
Everyday Health follows strict sourcing guidelines to ensure the accuracy of its content, outlined in our editorial policy. We use only trustworthy sources, including peer-reviewed studies, board-certified medical experts, patients with lived experience, and information from top institutions.
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Igor Kagan, MD

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Igor Kagan, MD, is an an assistant clinical professor at UCLA. He spends the majority of his time seeing patients in various settings, such as outpatient clinics, inpatient rounds,...

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Stephanie Watson

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Stephanie Watson is a freelance health writer who has contributed to WebMD, AARP.org, BabyCenter, Forbes Health, Fortune Well, Time, Self, Arthritis Today, Greatist, Healthgrades, ...