What Is IgA Nephropathy?
Immunoglobulin A (IgA) nephropathy, also called Berger’s disease, is an autoimmune disease that affects your kidneys.
IgA is an antibody. Antibodies are proteins made by your immune system. They protect your body from bacteria and viruses. When you have IgA nephropathy, some of the IgA your body makes has less of a particular type of sugar than typical IgA. Researchers believe this causes other antibodies to see them as foreign invaders and attack them, creating larger clumps of antibodies. Those bigger clumps build up in your kidney filters (glomeruli), causing inflammation and damage. This makes it harder for your kidneys to work properly.
Early detection and treatment can limit the harm IgA nephropathy can do to your kidneys. Without effective treatment, IgA nephropathy can cause permanent scarring in your kidneys over time. If your kidneys can no longer filter your blood well, they can’t keep the correct chemical balance in your body needed for healthy cells, tissues, and organs. As this scarring worsens, some people with IgA develop end-stage kidney disease (also known as end-stage renal disease, or ESRD) and need dialysis or a kidney transplant.
Only about 200,000 people in the U.S. have IgA nephropathy. You can get it at any age, but symptoms usually happen before age 40.
IgA Nephropathy Causes
Autoimmune diseases cause your body’s natural defense system (the immune system) to attack your normal cells and systems as if they are germs. It forms antibodies to help in this defense. When you have IgA nephropathy, your blood has a higher level of an abnormal type of IgA, an antibody that lacks a special sugar molecule. Other antibodies then target this IgA and form clumps that get stuck in the filtration system of your kidneys and damage it.
Scientists don’t know what triggers IgA nephropathy. It’s not contagious. Your genes play a role in whether you get it. The disease can run in families, so you may get it if one of your relatives has it or had it. But the disease also tends to have a trigger that sets it off.
These triggers can include:
- Liver diseases. Certain conditions affecting the liver, such as cirrhosis, and chronic hepatitis B and C infections, may be linked to setting off IgA nephropathy.
- Celiac disease. If you have this digestive problem, your body can’t process gluten, a protein found in most grains.
- Infections. HIV and some bacterial infections are linked to an increased risk of IgA nephropathy.
It’s not common for people with IgA nephropathy to have a family history of kidney disease. Instead, they are more likely to have a family history of diseases involving the immune system, such as psoriasis or asthma.
You can get IgA nephropathy at any age. It’s more common to get a diagnosis when you’re between the ages of 13 and 40. Nearly twice as many men get it than women. It’s more common in people who are Asian or White than other races. It’s rare to get it if you have African heritage.
IgA Nephropathy Symptoms
“One of the problems with IgA nephropathy is that there are not a lot of signs and symptoms someone would notice early in the disease,” says Brad H. Rovin, MD, director of the Division of Nephrology at The Ohio State University Wexner Medical Center. “One exception is patients who develop blood in their urine when they get a cold or upper respiratory infection.” Your urine may look tea-colored due to blood (hematuria), and it may also appear foamy if there is significant protein in the urine (proteinuria). And it can go back to looking normal when the infection clears up.
Many people have no symptoms, even for years. But in addition to blood or protein in your urine, you may have:
- Swelling in your ankles
- High blood pressure (hypertension)
Very rarely, someone may notice a lot of blood in their urine, making it look red.
If you have IgA nephropathy for a long time, it raises your risk of complications such as:
- High blood pressure
- Kidney failure that comes on suddenly
- Losing kidney function slowly over time (chronic kidney failure)
- Nephrotic syndrome, which is a group of symptoms including high protein in your urine, swelling, and high cholesterol
- Heart or cardiovascular problems
Diagnosis and Testing
Because there are few symptoms in the early stages of the disease, “early detection relies on finding abnormalities in the urine that the patient would not see — protein or blood only detectable under the microscope,” says Rovin. Your doctor will test your urine to see if there is blood or protein there, but those are only signs that your kidneys are not working well, which can happen with many different conditions. The only sure way to know if you have IgA nephropathy specifically is for a doctor to do a kidney biopsy.
Your doctor will perform a kidney biopsy in a hospital or an outpatient center by inserting a biopsy needle through your back to remove a small tissue sample. They’ll numb the area and may even sedate you. Your doctor will use imaging such as a CT scan to help guide the needle to the correct spot.
After the biopsy, you’ll spend about four hours in recovery lying down. Your doctor will advise you not to do a lot of physical activity for the next week and will send the sample to a doctor called a pathologist who looks at it under a microscope. There, they can see if the IgA has built up in your kidney tissue and, if so, how much damage there is. A biopsy can confirm your diagnosis and also help your doctor better decide which treatment to choose for you.
Once you have a diagnosis of IgA nephropathy, your doctor may want you to do testing from time to time to see how well your kidneys are working.
Blood and urine tests can check for problems such as:
- Anemia (a low red blood cell count)
- Bone disease
- Heart and blood vessel disease
- Acidosis (the buildup of acid in the blood)
My Diagnosis Story
John Marsala, assistant executive director, clinical trial engagement, IGAN Foundation, Westbury, NY.
My diagnosis came as a total surprise after a routine physical when I was 30. I had high levels of protein in my urine, and my primary care doctor was concerned enough that he sent me to a kidney specialist. The nephrologist told me he strongly suspected I had something called IgA nephropathy, but he couldn’t confirm it without a biopsy. After hearing the risks of the procedure, I decided not to go through with it, and instead, we waited to see how things unfolded.
The whole situation was out of the blue for me, but when I look back with the knowledge I have now, I can see I had symptoms. I was tired and my urine was foamy. But I was newly married and working two jobs. I just thought I was tired. I didn’t realize those weren’t normal.
Over the next month, things continued to get worse, and the doctor finally recommended the biopsy. This confirmed the diagnosis and got me started on treatment, which was just blood pressure medication and a small dose of prednisone. At this point, I wasn’t fully aware of the impact the disease would have on me. But soon after starting treatment, I applied for life insurance and was rejected. That was shocking to me because I had been thinking of my IgA nephropathy as something I just needed to take meds for, not a more serious condition that could potentially lead to end-stage renal failure, dialysis, or transplant.
Then I had a flare-up. My doctor put me on a high dose of prednisone, which unfortunately had every side effect possible, from insomnia to extreme weight gain to moodiness. About four months into that, he said it was time for me to find a specialist just for IgA nephropathy. Under the care of that doctor, I weaned off the prednisone and eventually, a couple of years later, entered a drug trial.
I was having good results from the trial. My protein was cut in half, and my GFR (glomerular filtration rate) stabilized. But about nine months into it, COVID-19 hit, and I got it. My health took a hit, and unfortunately, it put me in a place where I couldn’t recover and needed a kidney transplant. I received a new kidney a year ago. It doesn’t mean I’m cured — there’s a chance the disease could come back.
My health right now is good. Unfortunately, I had COVID two weeks ago, so my doctors are monitoring my blood work to see if that’s affected my kidney in any way. But I’m also immersed in my work with the IgA Nephropathy Foundation as their social media and clinical trial engagement director. I help people get into clinical trials, talk with them, and talk with pharmaceutical companies to help fill their trials. I’ve done a great deal through the foundation, including talking to Congress, sitting in front of the FDA on panels, and running support groups for the newly diagnosed as well as those continually living with the disease.
Meet the Care Team
When you’re dealing with IgA nephropathy, your medical team may include:
Primary care doctor. Your regular doctor may be the first person you go to if you are having symptoms and they may also be the first to diagnose you. They can monitor your health as you treat your IgA nephropathy, but they may also refer you to specialists.
Nephrologist. These specialists treat kidney disorders and diseases. Nephrons are the filtering units in your kidneys. Your regular doctor may refer you to a nephrologist for careful monitoring of your disease.
Nutritionist or dietitian. Your doctor may want to put you on a low-salt diet. This reduces the stress on your kidneys and heart. A nutritionist or dietitian can help you adjust your meals to adjust to your new eating plan.
Questions for Your Doctor
Before you go to the doctor, make a list of any and all symptoms you’ve been having and for how long. Also, write down all the medications and supplements you’re taking, along with dosage information.
When you visit your doctor, gather as much information as you can so you’re in the know about your disease and how to manage it. Bring a trusted friend or loved one with you who can take notes and help you remember what you learn.
You can ask:
- How advanced is my condition?
- What medications do you recommend?
- Should I eat a special diet?
- What symptoms should I be on the lookout for?
- How often will I have blood and urine tests?
- What kinds of doctors should I see?
- Do I need a kidney transplant, or might I need one in the future?
- What other lifestyle changes can I make to take care of myself?
- How might my other health conditions affect my IgA nephropathy?
- What is the best way to get in touch with you if I have a question?
- Where can I look for more information about my disease?
- Are there any clinical trials available?
Your doctor will have questions for you as well, such as:
- Do your symptoms happen all the time or only occasionally?
- How severe are your symptoms?
- Does anything seem to improve your symptoms?
- Does anything make your symptoms worse?
IgA Nephropathy Treatment
Currently, there is no cure for IgA nephropathy, but “there have been a lot of new medications developed and approved for the treatment of IgA nephropathy that seem to control protein loss and help decrease the fall in kidney function over time,” says Rovin. “These include drugs that act on the pathways that result in formation of abnormal IgA molecules, drugs that decrease inflammation in the kidneys, and drugs that act within the kidney to slow disease progression.”
Clinical trials of some drugs that slow the progressive decline in people with other types of chronic kidney diseases may show promise for people with IgA nephropathy.
These meds can:
Reduce the protein in your urine. Two newer medications that do that are sparsentan (Filspari) and iptacopan (Fabhalta). Sparsentan blocks a receptor that can reduce blood flow in the kidney. Less blood flow means waste is not efficiently filtered, leading to protein in the urine. One advantage of sparsentan over other medications for IgA nephropathy is that it does not suppress your immune system, lowering your risk for infections. Iptacopan acts on a specific process in the kidneys that, when disrupted, can release more protein into the urine.
Control blood pressure. Medications such as angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) help manage high blood pressure and protect your heart health. This can also slow the advance of kidney disease. You may also need other medications such as beta-blockers or calcium channel blockers if your blood pressure is still too high.
Remove extra fluid from your blood. Diuretics are medications that help get extra fluid out of your blood when your kidneys can’t. Your doctor may prescribe this along with blood pressure medications.
Manage your immune system. To help reduce inflammation in your kidneys, you may need a steroid medication such as prednisone, or a medication called cyclophosphamide that slows down or dampens your immune system. It is not entirely clear if the benefits of these medications outweigh the risks, and who should get them. A newer medication called Nefecon (Tarpyeo/Kinpyego) can reduce inflammation with fewer side effects than other steroids.
Lower your cholesterol. Statins are medications that help keep high cholesterol in check. It’s common to have higher cholesterol levels when you have IgA nephropathy.
Tonsillectomy for IgA nephropathy
A tonsillectomy is surgery to remove the tonsils. It is used to treat IgA nephropathy in Japan. Tonsils produce antibodies, including the IgA molecules that have less sugar and cause IgA nephropathy. By removing the tonsils, doctors aim to lower the amount of abnormal IgA formed in people with IgA nephropathy.
“The data from clinical studies showing that this is beneficial is very limited,” says Jonathan Barratt, MD, The Mayer Professor of Renal Medicine at the University of Leicester in the United Kingdom. “There are no data to support the use of tonsillectomy as a treatment for IgA nephropathy outside of Japan.” Currently, U.S. and international guidelines do not recommend tonsillectomy to treat IgA nephropathy.
Living With IgA Nephropathy
Along with regular checkups with your doctor to monitor your kidneys and other symptoms, you can do some things at home to boost your kidney health:
- Boost your intake of omega-3 fatty acids. You can get these healthy fats by taking fish oil supplements and eating fish. They may help reduce inflammation in the glomeruli and don’t cause harmful side effects. You can buy supplements over the counter, but talk to your doctor before taking them.
- Keep your blood pressure in check. Having a healthy blood pressure can help slow down kidney damage. Take your medications the way your doctor prescribes. You can also improve your blood pressure through diet, especially by reducing your salt intake and avoiding excess alcohol. Losing excess weight and getting regular exercise are also helpful. You should also keep track of your blood pressure numbers by monitoring them at home.
- Eat less protein. Eating too much protein can make your kidneys work too hard and speed up the worsening of your IgA nephropathy. But before you cut back, check with your doctor or nutritionist to be sure you get enough. Eating too little protein can cause malnutrition.
- Eat less salt. Too much salt can increase your blood pressure and cause more protein to spill into the urine.
- Avoid harmful meds. This can include over-the-counter meds such as NSAIDs, as well as certain prescription medications. Talk to your doctor before starting any new medicine.
- Quit smoking. In addition to slowing down blood flow to your kidneys, smoking can affect how well your blood pressure medications work.
- Drink the right amount of water. Help your body work well with the right amount of fluids. But talk to your doctor about how much is too much.
Caring for Your Emotional and Mental Health
Dealing with a chronic illness, such as IgA nephropathy, can take a toll on your mental health. Physical and mental health are linked. Here are some steps you can take to protect your emotional wellness as you manage your disease.
Advocate for yourself. Ask the questions you need to ask. Speak up when you’re worried or unsure about something. Make sure you have a medical care team that is listening to you and that you feel comfortable with.
Find a counselor. A mental care professional is a great resource for talking through your emotions and struggles. You can find someone through your insurance or ask your doctor for recommendations.
Keep doing what you love. Nurture hobbies and passions when you can. Do things that are fun and bring you joy.
Manage stress. Practice yoga, meditation, or breathing exercises to help your body feel relaxed and grounded.
Stay on top of your care. Don’t miss doctor appointments. Track your symptoms and overall health so you’re an active part of your care.
What to Expect
There are several ways your IgA can progress, including:
- It can go away completely (more common in kids).
- Kidneys may slowly lose function and require monitoring.
- Kidneys may stay the same for many years and require only regular checkups.
- It may lead to kidney failure, requiring dialysis or a kidney transplant.
Your treatment and outlook will depend on several things, including:
- Your age
- Your overall health and past health
- The amount of protein in your urine (more protein means the disease is getting worse)
- Your kidney’s filtration rate, also called glomerular filtration rate (GFR)
- The information your doctor gets from your biopsy, including the extent of scarring
- How well you can handle specific medicines, procedures, or therapies
IgA nephropathy life expectancy
IgA nephropathy is a disease that progresses slowly for most people. The goal of early detection and treatment is to keep your kidneys working well and avoid kidney failure. Research suggests that 20%-50% of people with IgA nephropathy will eventually develop kidney failure and need dialysis or a kidney transplant. That means many do not. Some studies have found that IgA nephropathy can decrease life expectancy, but with early detection and effective treatment, many people can live long lives while staying engaged in work as well as other important and enjoyable activities.
Where to Find Support
There are online and in-person resources that can help you connect and feel less alone as you navigate life with IgA nephropathy. Some of the organizations you can turn to for information and support include:
- National Kidney Foundation. Its NKF Peers group is a national telephone-based peer support program.
- National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). It not only offers educational information about IgA but also has a Community Health and Outreach section.
- IgA Nephropathy Foundation. This nonprofit public health organization helps fund research about IgA nephropathy.
Your doctor may also know of in-person support groups in your area.
Takeaways
Immunoglobulin A (IgA) nephropathy, also called Berger’s disease, is a rare autoimmune disease that affects your kidneys. Early signs of IgA nephropathy include blood and proteins detected in urine, but it can only be diagnosed with a kidney biopsy. If you notice your urine is tea-colored, cola-like, or reddish, see your health care provider right away. Early treatment can help you live a long life and reduce the need for dialysis or a kidney transplant.
IgA Nephropathy FAQs
What are the stages of IgA nephropathy?
The National Kidney Foundation developed stages of chronic kidney diseases, such as IgA nephropathy, that are based on two pieces of data. The first is called the glomerular filtration rate and is measured by a blood test that shows how much creatinine is in your blood. Creatinine is a waste product that your kidneys filter out. The more creatinine in your blood, the less well your kidneys are working. The other test is called an albumin-to-creatinine ratio (uACR) and is measured by a test that checks how much albumin (a protein) you have in your urine. Like creatinine in your blood, higher levels of albumin in your urine suggest poorer kidney function.
- Stage I: Kidneys are only slightly impaired and can function normally. You probably don’t have symptoms.
- Stage II: Kidneys are only slightly impaired and still work well, and you may not have symptoms.
- Stage III: Kidneys develop mild to severe impairment and do not work as well as they need to. You might start to feel symptoms such as tiredness, feeling weak, or having swelling in your hands or feet.
- Stage IV: Kidneys are moderately to severely damaged and are close to stopping working. You will probably feel symptoms such as swelling in your hands and feet and back pain, and you are at risk of developing high blood pressure and bone or heart disease.
- Stage V: Kidneys are the most impaired and are close to not working at all or have stopped working. This is when people feel very sick and will need dialysis or a kidney transplant.
What foods should you avoid with IgA nephropathy?
Before making any changes to your diet, talk to your doctor. Generally speaking, if you have IgA nephropathy, you should cut back on salt (as it makes it harder for your kidneys to work). Also, lower your protein intake, particularly from animal products. Everyone is different, but people with kidney disease are advised to consume less than 3/4 of a teaspoon of sodium (or 1,500 milligrams) a day. Ask your doctor what they recommend, and read the labels of packaged foods, which often have a lot of salt. There are other changes you can make that may help keep you healthy or help you manage or avoid other conditions such as high blood pressure.
Can I live a long life with IgA nephropathy?
Yes, with effective treatment, you can live a long life and continue doing the things you need and love to do.
Can IgA nephropathy be reversed?
No, there is no cure for IgA nephropathy, but new medications that significantly improve treatment have recently been approved or are being studied now.