IgA Nephropathy (IgAN)

What are the signs and symptoms of IgA nephropathy?

Kidney damage from IgA nephropathy can take years to develop. Some people with IgA nephropathy do not have any symptoms for 10 years or more, and some people can have IgA nephropathy and not know it. Others can have a more aggressive version that is diagnosed more quickly.

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
• Have swelling in your hands or feet or around your eyes

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 

How can doctors tell if I have IgA nephropathy?

IgA nephropathy is diagnosed using a kidney biopsy. Your doctor will first test your kidney function using urine and blood tests.  

Urine testing: 

uACR: (albumin-to-creatinine ratio) is a urine test that measures the amount of albumin (a type of protein) in your urine compared to the level of creatinine (a waste product). This ratio helps your doctor determine how well your kidneys are working. 

The uACR test can detect early-stage kidney damage, especially for people at a higher risk. A higher uACR means more kidney damage and can indicate your risk for quick progression to kidney failure. For IgAN patients, this is crucial in helping to monitor kidney function and understanding how quickly the disease can progress.

uPCR: (urine protein-to-creatinine ratio) is a urine test that measures total protein in the urine. 

Blood testing: 

eGFR (estimated glomerular filtration rate) is a blood test that shows how well your kidneys are working by measuring the waste and toxins in your blood. It typically uses how much creatinine is in your blood to calculate it. Creatinine is a waste product from your muscles that should be filtered out by your kidneys; if your kidneys are damaged, it can build up in your blood.

Kidney biopsy 

A kidney biopsy is a procedure where doctors take a small piece of tissue from your kidneys to look at it under a microscope. With IgAN, a kidney biopsy will show deposits of IgA in the glomeruli of your kidneys. This is the only definitive way to diagnose IgA nephropathy. 

If you have over 0.5 grams of protein in your urine a day, your doctor may suggest a kidney biopsy. The amount of protein in your urine is measured with a urine protein-to-creatinine ratio (uPCR) test. 

The most common type of kidney biopsy is called needle biopsy. In needle biopsy: 

• You will be awake during the procedure. 
• You will get medicine to help you relax and numb the area. 
• Your doctor will guide a needle device into your kidney. 
• Then they will take a small sample of kidney tissue. 

Rarely, doctors do an open biopsy, which is a surgery with medicine to put you to sleep (anesthesia). Ask your doctor about an ultrasound or CT-guided needle biopsy. This uses imaging to help guide the biopsy needle to lower the chance they will hit a major blood vessel and cause severe bleeding.

How do doctors treat IgA nephropathy?

There is no cure for IgA nephropathy. However, there are treatment options to lower the build up of IgA and inflammation in your kidneys. This helps slow down damage to your kidneys to prevent kidney failure, dialysis or a kidney transplant.

You and your doctor will work together to create a treatment plan that works best for you. You will likely be on multiple medicines that help protect your kidneys in different ways.

There are two types of medicines that specifically treat IgAN: medicines that lower the amount of protein in your urine and medicines that lower the amount of IgA antibodies or block inflammation. They help reduce harmful inflammation, which helps block the different ways IgAN damages your kidneys.

Medicines that lower the amount of protein in your urine and slow kidney decline:

• Endothelin receptor antagonists, atrasentan (Vanrafia®), block the action of a hormone called endothelin. This helps reduce inflammation that causes damage to the kidneys over time. 
• Dual endothelin angiotensin receptor antagonists, sparsentan (Filspari®) work in two ways: 
  • Lower your blood pressure and the pressure inside the kidneys' tiny filters. They can replace an ACE inhibitor or ARB. 
  • Block the effects of two hormones (endothelin and angiotensin) from triggering inflammation.

Medicines that lower the amount of abnormal IgA antibodies or block IgA inflammation:

• Targeted-release budesonide (e.g., Nefecon®, Tarpeyo®), is a type of steroid that is released in the intestines, so it targets cells that make IgA. This helps lower the buildup of IgA in your kidneys. 
• Complement blockers, iptacopan (Fabhalta®),  block part of the complement system pathway, which is an immune response that can be triggered by IgA that is built up in your kidneys. This lowers inflammation in your kidneys from the immune response. 
• APRIL blockers, sibeprenlimab-szsi (Voyxact®), block a protein called APRIL (which stands for A PRoliferation-Inducing Ligand) that helps make IgA. This helps lower the buildup of IgA in your kidneys. 
• Dual APRIL/BAFF blockers, atacicept (Trutakna®), block APRIL and another protein called BAFF (which stands for B-cell Activating Factor), which help make IgA. This helps lower the buildup of IgA in your kidneys.

Steroids are another treatment option that may be recommended. They suppress your immune system which can help remove inflammation in the body including your kidneys.

• Talk to your doctors to see what options may be best for you. There are also ongoing clinical trials that are testing how well other treatments work which may be FDA- approved and available for prescription in the future.

In addition to these medicines, your doctor may also recommend that you make lifestyle changes such as: 

• Follow a kidney-friendly eating plan, which includes changes in what you eat to lower the amount of protein, fat, sodium and cholesterol you take in
• Keep a healthy weight 
• Be physically active most days of the week, for at least 30 minutes
• Stop any tobacco use, including cigarettes and vaping
• Keep your blood pressure lower than 120/70 mmHg. Lifestyle changes and medicines such as ACE inhibitors, ARBs, or SGLT2 inhibitors can help you reach this goal.

Your doctor will also do regular tests to keep track of your kidney health over time. With these measures, many people will see a reduction in the amount of protein that is in the urine. 

What is the 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.

What can I expect while living with IgAN?

Just Diagnosed 

You may have been struggling with chronic kidney disease (CKD) for a long time before your IgA nephropathy diagnosis, or you may have discovered your kidney damage and IgA nephropathy at the same time. 

In both cases, this diagnosis can be overwhelming. IgA nephropathy is a chronic disease, meaning you will have it for the rest of your life. Over time, the disease can get worse. IgA nephropathy causes kidney failure in about half of all patients within 10 years of diagnosis. 

Talk to your doctors early on and often about treatment options, clinical trials, and any new symptoms to help prevent future damage.

IgA Nephropathy Progression 

IgA nephropathy is a lifelong, progressive disease, meaning it will gradually worsen over time. For some people, the progression may be slow, and they may never reach kidney failure. However, for others, the disease can progress quickly, leading to kidney failure in just a matter of months. One key warning sign is proteinuria (protein in the urine). The more protein you have in your urine, the higher your risk of quick progression to kidney failure.

It is possible for IgAN to reoccur after a kidney transplant. It often happens many years after a transplant and does not always cause signs of disease. Researchers are not sure how often recurrence of IgAN leads to rejection of a transplanted kidney, but a high level of proteinuria and scarring in the transplanted kidney are both associated with a higher likelihood of rejection.

A common test to measure proteinuria is the urine albumin-to-creatinine ratio (uACR). This is a urine test that uses a small amount of urine to compare how much albumin (protein) to creatinine (waste product) is in your urine. This test can indicate your level of kidney damage. 

Proteinuria levels: what do they mean? 

Having any amount of protein in your urine can be a sign of kidney disease. If you have IgAN, the amount of protein in your urine can indicate how likely you are to progress towards kidney failure. 

Talk to your doctor about your most recent uACR test results and what the results may mean for your kidney disease.

uACR Results:

• Normal to mildly increased <30 mg/g or <3mg/mmol
• Moderately increased: 30-299 mg/g or 3-29mg/mmol
• Severely increased: ≥300 mg/g or ≥30 mg/mmol

If you have not had a uACR test before, talk to your doctor about getting one done and incorporating the test into your monitoring strategies. 

Since you may not have immediate signs of proteinuria in the early stages, it's important to talk to your doctor about regularly monitoring your urine and blood levels and ways you can slow damage to your kidneys. 

If you are close to kidney failure, it's important to have open conversations with your healthcare team about treatment options and what is best for you. Treatments available for kidney failure include dialysis (hemodialysis or peritoneal dialysis) and a kidney transplant. 

Proteinuria Symptoms

While there are not always symptoms of proteinuria, the following can indicate high levels of proteinuria: 

• Swelling in your face, belly, feet or ankles
• Urinating (peeing) frequently
• Shortness of breath
• Being tired frequently
• Nausea or vomiting
• Lack of hunger
• Puffy eyes
• Foam or bubbles in your urine 

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. 

Find healthy ways to cope 

It is normal to have stress, anxiety and depression when facing difficult situations. If you notice changes to your mental health, these activities may help you feel better: 

Be active for at least 30 minutes most days of the week. Exercise can lower feelings of depression and anxiety and improve the way you think, learn and remember things.

Get enough sleep. Aim for 7-8 hours a night and try to go to sleep and wake up at the same time every day. Poor sleep affects mood, energy, attention span and your ability to think clearly.

Tap into your faith. For some, connecting with religion and spirituality may serve as a valuable way to cope with mental health issues. Your faith community can provide a group of people that you can rely on when you are feeling down.

Explore your creative side. Pick up an old hobby or try something new such as painting, crafting or sewing. When engaged in something you enjoy, it can lower stress and release feel-good chemicals in your brain. 

Talk to a professional 

If you want to talk to a professional, ask a member of your treatment team for a referral to a mental health therapist, counselor or social worker. 

These professionals are trained to help you: 

• Understand and process your emotions
• Improve your coping skills 
• Maintain healthy relationships with loved ones 
• Navigate everyday life with chronic disease
• Advocate for your needs 

Many health insurance providers cover mental health services. Call your health insurance provider to find out what your health insurance plan includes and get a list of in-network mental health professionals in your area. 

Find a support group 

Having a chronic disease like IgA nephropathy can feel isolating. But you are not alone. One of the best ways to cope with a major life change is to connect with people who are going through a similar experience.   

Benefits of joining a support group include: 

• Being able to talk openly and honestly with people who understand what you are going through
• Learning tips for managing IgA nephropathy from people who have been living with it for longer than you
• Learning more about IgA nephropathy and opportunities for patients, such as clinical trials and new treatments
• Finding new ways to cope
• Feeling empowered and supported by your new community
• Making new friends 

Visit the IgA Nephropathy Foundation and National Organization for Rare Disease (NORD) to learn more about support groups and other community-based resources near you.