The Stages of IgA Nephropathy (Berger's Disease)

Immunoglobulin A nephropathy (also known as IgA nephropathy or Berger's disease) is a condition that causes IgA antibodies to accumulate in the kidneys—leading to damage that affects their blood-filtering function. Each stage of this condition affects your kidneys a bit differently.

There are five general stages of chronic kidney diseases like IgA nephropathy. Each stage indicates how well your kidneys are functioning.

It's worth noting that the progression of IgA nephropathy can be slowed down or prevented, so seeking a diagnosis when you start experiencing symptoms is essential. While the course of the disease can vary by person, IgA nephropathy typically progresses slowly for 10 to 20 years—and not all cases will reach the kidney failure stage.

How Do Providers Determine What Stage You're In?

A healthcare provider can perform several tests to diagnose IgA nephropathy and determine the stage of the condition by assessing how well your kidneys are functioning.

Here's what diagnostic exams your healthcare provider will typically order:

• Blood tests: A blood creatinine test can measure the severity of kidney disease, while a blood urea nitrogen (BUN) test can help evaluate kidney function. Another blood test called estimated glomerular filtration rate (eGFR) will estimate how well your kidneys remove waste products from the blood.
• Urine tests: A general urine test (urinalysis) determines whether there's blood or protein in your urine, a telltale sign of IgA nephropathy. A urine protein-creatinine ratio (PCR) test will also measure the levels of waste (protein and creatinine) to assess kidney damage.
• Kidney biopsy: To confirm an IgA nephropathy diagnosis, a sample of your kidney will be removed during a kidney biopsy. This shows the amount of the IgA antibody present and indicates how much damage has been done to your kidneys.

IgA nephropathy has a genetic component, meaning it tends to run in families. A healthcare provider will likely ask if you have a family history of the condition to help support your diagnosis and treatment.

IgA Nephropathy Staging System

The National Kidney Foundation (NKF) utilizes a staging system for several chronic kidney diseases, including IgA nephropathy.

These guidelines are divided into five stages based on the glomerular filtration rate (GFR), a formula that considers factors like age, assigned sex at birth, and creatinine levels in the blood. Fully functioning kidneys remove creatinine (a waste product produced by the body) from your blood, so its levels rise as kidney function diminishes.

IgA nephropathy is typically a slowly progressing disease. How quickly kidney damage occurs can vary based on individual factors, including when you were diagnosed, the treatment you're receiving, and any other underlying health conditions that could affect the kidneys, like diabetes.

Here's a brief overview of what each stage of IgA nephropathy entails:

Stage 1

In stage 1 of IgA nephropathy, you'll still have normal kidney function—but mild kidney damage is starting to occur.

You likely won't have noticeable symptoms, but you may have recently experienced a cold or upper respiratory infection that triggered the onset of IgA nephropathy. If blood is present in your urine, it probably won't be visible to you but can be detected during a urine test. A urine test may also reveal protein levels in your urine that suggest chronic kidney disease.

If you receive a diagnosis of IgA nephropathy at this early stage, there is time to slow kidney damage and maintain your current kidney function. Your healthcare provider will likely focus on helping you:

• Maintain healthy blood pressure
• Control blood sugar levels, particularly if you have diabetes
• Weigh the options for prescription medications to protect against kidney damage
• Manage weight through a nutritious, low-sodium diet and plenty of exercise

Stage 2

There is a mild loss of kidney function as you reach stage 2 of IgA nephropathy. However, the kidneys still work relatively well, and you may not see many noticeable signs. You may potentially see some blood in your urine, and you might experience slight swelling in the hands and feet as fluid builds up in the body.

A urine test will probably reveal higher-than-normal protein levels. If your healthcare provider orders an imaging scan (like an ultrasound), visible kidney damage may be present. A kidney biopsy can confirm this diagnosis.

In these early stages, your provider will want to focus on avoiding complications from IgA nephropathy, such as high blood pressure or diabetes, and may suggest the following:

• Maintaining healthy blood pressure and blood sugar levels
• Taking medications (like statins) to help protect against heart disease and further kidney damage
• Considering the pros and cons of medications that target the immune system directly (like corticosteroids) before too much kidney damage has occurred
• Recommended lifestyle changes, like eating a kidney-friendly diet and avoiding smoking

Stage 3

In stage 3, mild to moderate kidney damage is occurring. Your kidneys aren't functioning as well as they should and are unable to remove waste and excess fluid from your blood as normal.

Some people start to experience noticeable symptoms at this time, like apparent swelling in the hands and feet or feeling weak and tired. Other health concerns may start to develop as waste builds up in the body, like high blood pressure.

You will probably not progress to later stages if you receive a diagnosis at stage 3 and start treatment. There is still damage to your kidneys at this stage, so a healthcare provider will want to treat the symptoms of IgA nephropathy and avoid disease progression.

They can generally prevent the disease from worsening by prescribing medications such as:

• Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs): Control high blood pressure and swelling and reduce protein levels in the urine
• Corticosteroids: Suppress an overactive immune system and reduce inflammation
• Statins: Lower cholesterol levels and prevent heart disease from developing 

Lifestyle modifications will still be important during this period, so you may meet with a nutritionist to discuss a diet low in sodium and moderate in protein intake. They might also recommend limiting your fluid intake to control further inflammation.

Stage 4

Moderate to severe damage to the kidneys is occurring during stage 4. Your kidneys are not working well to effectively filter waste from your blood.

If they haven't yet, symptoms like swelling of the hands and feet or lower back pain typically manifest during this time. Other health conditions like high blood pressure and heart disease may also develop as the waste builds up in your body.

Because this is the last stage before kidney failure, treatment from a nephrologist (a doctor who specializes in treating kidney disease) is necessary to slow the damage and assess the potential for kidney failure. They may recommend the following procedures:

• Dialysis: Uses a machine to manually remove waste from the blood several times per week
• Kidney transplant: Replaces a damaged kidney in your body with a healthy donor kidney

While these treatment options can help add years and quality of life to your prognosis, note that neither of these procedures is a cure for IgA nephropathy. After a kidney transplant, it's still possible for your new kidney to become damaged, too.

Stage 5

There is severe damage to the kidneys in the last stage of IgA nephropathy. This stage significantly increases your risk of kidney failure, where the kidneys can no longer function. You're likely feeling very ill due to the buildup of waste in the body, and possibly experiencing other health concerns. Some of the key symptoms during this stage include:

• Trouble breathing
• Producing little or no urine
• Lack of appetite
• Severe swelling of your feet and hands

Kidney failure can become life-threatening if left untreated, so your healthcare provider should pursue an aggressive treatment strategy to prevent this from occurring.

It would be considered rare to reach this stage within a few years of an IgA nephropathy diagnosis, as this condition is usually slow to progress.

Around one in five people with IgA nephropathy develop kidney failure within a decade. Additional data suggests that somewhere between 20% and 50% of people with IgA nephropathy will reach kidney failure within 20 to 30 years of being diagnosed with the condition.

Preventing Progression

While IgA nephropathy is not curable, an early diagnosis and the right treatment strategies make it possible to delay or prevent the progression of the disease. Data suggests that up to half of IgA nephropathy cases may progress so slowly that they don't get worse over time.

Follow your treatment plan closely and discuss it with your healthcare provider if you have any questions or concerns. Research shows that prescribed medications for IgA nephropathy help control blood pressure and reduce the amount of protein in urine, ultimately slowing or preventing damage to the kidneys.

Your healthcare provider may also recommend lifestyle changes to support this goal, including:

• Eating a low-sodium diet to reduce inflammation and manage high blood pressure or cholesterol levels
• Monitoring your fluid intake to reduce overall inflammation in the body
• Exercising regularly to maintain an ideal weight for your body
• Quitting or avoiding smoking to help slow kidney damage and lower kidney failure risk

Life Through the Stages

The stages of IgA nephropathy can affect people in different ways. Some people don't experience many noticeable symptoms or adverse health effects, while others may live with severe symptoms and go on to develop kidney failure.

The early stages of IgA nephropathy may not entail intense symptoms. One notable sign may be experiencing blood in your urine after a cold or upper respiratory infection, which experts have found to be a trigger for the development of IgA nephropathy.

Other early symptoms may include mild fatigue and pain in the lower back. During this time, it's important to implement lifestyle changes that help lower the risk of additional health concerns.

As IgA nephropathy progresses, symptoms can affect your quality of life and complications may start to occur. This might include swelling and fatigue that interrupt your daily routine. A healthcare provider will work with you on a medication treatment plan to mitigate symptoms and minimize further damage to your kidneys.

During the later stages of IgA nephropathy, when symptoms are severe and you require treatments like dialysis or a transplant, your quality of life may be severely affected. This can be challenging to experience, so it's important to lean on your healthcare team and loved ones for support.

Many people experience the extreme stress of living with a chronic disease. This can contribute to mental health conditions like anxiety and depression. Managing your stress levels, particularly through mindfulness techniques like yoga and meditation, has been shown to help improve depression symptoms, heart and lung function, and overall quality of life in people with IgA nephropathy and chronic kidney disease.

A Quick Review

IgA nephropathy occurs when an immune system response causes IgA antibodies to build up in the kidneys—leading to damage that makes it difficult for the kidneys to filter waste from your blood. Healthcare providers use a staging system to determine the stage of this chronic kidney disease (stages 1 through 5) before reaching kidney failure.

The symptoms you experience and their severity will likely depend on your individual health and the stage of the disease. Treatment plans that include prescription medications, lifestyle changes, and potential surgical procedures can help slow or prevent the progression of IgA nephropathy through the stages and improve your quality of life.