Immunoglobulin A (IgA) nephropathy is a rare disease caused by a buildup of abnormal IgA antibodies in the kidneys. Harmful inflammation occurs in the kidneys, which, over time, leads to kidney damage or even end-stage renal disease (ESRD).
There is no cure for IgA nephropathy, but early diagnosis and appropriate treatments can help slow damage to the kidneys.
Key Points
- IgA nephropathy is a type of kidney disease in which the IgA protein builds up in the kidneys, causing inflammation.
- The most common symptoms are hematuria (blood in the urine) and proteinuria (protein in the urine). While seeing blood in the urine can be alarming, the presence of increased protein in the urine predicts which patients are at most risk of developing serious kidney disease.
- Urine and blood tests identify that there is a kidney disease. A kidney biopsy may be ordered to diagnose IgA nephropathy with certainty.
- The goal of treatment is to control blood pressure, inflammation, cholesterol and protect the kidneys from further damage.
What is Immunoglobulin A (IgA) Nephropathy?
Immunoglobulin A (IgA) nephropathy is a somewhat rare kidney disease where IgA proteins build up in the kidneys and form clumps, damaging the glomeruli (tiny blood vessels that filter blood). IgA are proteins made by the immune system. These IgA proteins are antibodies that typically protect the body from infection. This IgA accumulation causes inflammation in the kidney (glomerulonephritis) leading to damage to the glomeruli causing the kidneys to leak protein and blood into the urine. This damage also leads to scarring of the glomeruli and the rest of the kidneys which can lead to kidney failure.
In the U.S., as many as 1 in 10 kidney biopsies show IgA nephropathy.
Complications of IgA nephropathy
IgA nephropathy is a common cause of kidney failure and chronic kidney disease (CKD). Other complications of IgA nephropathy include:
- IgA vasculitis (A more severe version with inflammation of small blood vessels sometimes causing Acute kidney failure)
- Hypertension (high blood pressure)
- Nephrotic syndrome (Depleted protein in the blood and swelling caused by a severe protein loss in the urine)
- Heart or cardiovascular problems
- End-stage kidney disease requiring kidney transplant or dialysis
- Death
The Signs and Symptoms of IgA Nephropathy
It may take a few years or decades before kidney damage from IgA nephropathy develops. No two people with IgA Nephropathy are the same. Some people may not have any signs or symptoms of IgA Nephropathy, resulting in some people not knowing they have the disease. Other people with this disease can have an aggressive version that presents more quickly and is diagnosed earlier.
The first signs of IgA nephropathy are normally hematuria (blood in the urine) and proteinuria (too much protein in the urine). Symptoms of hematuria and proteinuria typically include:
- Foamy urine
- Pink, tea, or cola-colored urine, especially after a respiratory infection, cold, or sore throat
- Swelling in the feet, hands, or around the eyes
- Visible blood in the urine
Other common symptoms of IgA nephropathy may include:
- Edema, selling in the ankles
- Pain in the sides or lower back
- Hypertension (high blood pressure)
- Feeling tired or weak
What Causes IgA Nephropathy?
It is not known exactly what causes IgA nephropathy. Research suggests that the environment and genetics may play a role in this disease. An excess amount of IgA and/or abnormal structure of the IgA leads to IgA forming clumps (immune complexes) and then getting stuck in the glomeruli. Once these antibodies are in the kidneys, the immune system is triggered, damaging kidney tissue. Often IgA nephropathy is first identified due to blood in the urine following a sore throat, cold, or respiratory infection that increases the amount of IgA in the blood as the immune system fights the infection. IgA nephropathy can develop in any person; however, it is typically more common in people who:
- Are of Asian or Pacific Island ancestry (IgA is less common with European ancestry and least common with African ancestry)
- Are male
- Are between the ages of 10 and 40 years old
- Have human immunodeficiency virus (HIV)
- Have Celiac disease or inflammatory bowel disease (IBS)
- Have Cirrhosis (liver failure)
How is IgA Nephropathy Diagnosed?
In addition to reviewing symptoms, healthcare professionals will conduct a physical exam, take a medical history, and request tests.
To get a complete picture of your kidney health and determine if you have IgA nephropathy, the following tests may be used:
- Urine protein test to check for protein or albumin in the urine
- Blood urine nitrogen (BUN) test to measure kidney function
- A blood test to measure your glomerular filtration rate (GFR).
- Kidney biopsy to identify IgA deposits
- Physical exam, including blood pressure checks and check for signs of swelling
- Urinalysis to check for blood in the urine
As of now, the only definitive way to diagnose IgA nephropathy is with a kidney biopsy.
How is IgA Nephropathy Treated?
There is no cure for IgA nephropathy. Treatment aims to delay or prevent progression to chronic kidney failure and relieve and manage symptoms. Your healthcare provider will work with you to create an effective treatment plan.
Treatment may include:
- Medicines to lower cholesterol, such as statins. Controlling cholesterol can help lower the risk of developing heart attack or stroke.
- Blood pressure medications, such as Angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs), and dual endothelin and angiotensin Receptor blockers are used to manage and control high blood pressure and swelling. These medicines also help prevent protein loss in the urine.
- SGLT2i medications which decrease protein in the urine and protect the kidneys
- Corticosteroids and other medications suppress the immune system, reducing inflammation and scar tissue within the kidney.
- Prescription-strength fish oil to help reduce inflammation and kidney damage
It may be recommended to:
- Make diet changes, such as reducing salt, cholesterol and fat.
- Increase or decrease fluid intake
- Quit smoking
- Lose weight
Regular testing will be conducted to monitor kidney health over time. Talk to your healthcare provider about the latest research in IgA nephropathy.
Treatment for kidney failure from IgA Nephropathy
Kidney failure from IgA nephropathy may require dialysis or a kidney transplant. A kidney transplant is not a cure for IgA nephropathy. After a transplant, it is possible that the new kidney may be damaged due to IgA nephropathy.
Can IgA Nephropathy be Prevented?
There is no known way to prevent IgA nephropathy from developing. If this disease runs in your family, it is important to talk to your healthcare provider. Steps, such as managing your cholesterol and blood pressure, may be recommended.
Prognosis for people with IgA Nephropathy
There is no cure for IgA nephropathy. However, with the appropriate treatment, people can slow disease progression, keeping their kidneys healthy for as long as possible. Research has shown that medicines that manage blood pressure and prevent protein from moving into the urine can help slow kidney damage.
In some cases, IgA nephropathy may get better on its own. In rare cases, however, it progresses to kidney failure rapidly (within a few years). Roughly 1 in 4 adults with IgA nephropathy eventually develops end-stage renal disease (ESRD).
What can you do?
While there is yet no cure for IgA neuropathology, there is still a lot that you can do to help manage your disease and preserve kidney function. To get started, the IgA Nephropathy Foundation recommends the following steps:
- Find your community: These will be people who you trust to support you on your journey.
- Learn more about IgA Nephropathy: The testing protocols, treatment options, how to adopt a kidney-friendly lifestyle, and any signs and symptoms that you need to look out for.
- Connect with IgA Nephropathy support groups: You are not alone on your IgA nephropathy journey. Support groups allow you to connect with other IgA nephropathy patients and caregivers.
- Be active in your care and advocate for yourself: An effective way to manage IgA nephropathy is to keep track of your blood pressure and test results. Prepare a list of questions to ask your healthcare provider.
Clinical Trials
Clinical trials are conducted to find better, more targeted treatments for diseases such as IgA nephropathy. Participating in clinical trials not only provides you with the latest treatment and care, but it is also crucial to finding a treatment and, ultimately, a cure for IgA nephropathy.
Ask your healthcare provider or care team if there is a clinical trial that is a suitable option for you. To participate in a clinical trial, you will need to meet the study criteria, for example, in a certain age group, have a GFR within a certain range, or not be on a specific medication for a defined period of time. IgA Nephrology Foundation has a list of active clinical trials.
Panoramic Health
Panoramic Health is an integrated provider group delivering the future of kidney care. Our mission is to improve patients’ outcomes by slowing disease progression and improving their quality of life. We do this through the distinctive capabilities of our comprehensive care model, which brings together an integrated provider group with operational support, clinical support, and data & analytics at scale.
Through partnerships with practices, providers, payers, and health systems, we work to advance the usage of clinically validated best practices and cutting-edge data analytics across a continuum of reimbursement models.
*The content presented in this article is for informational use only and should not be construed as medical advice or replace the medical advice of a qualified physician.
