7 Warning Signs of IgA Nephropathy (Berger Disease) You Should Know

IgA nephropathy, also known as Berger’s disease, is a chronic kidney condition that occurs when an antibody called immunoglobulin A (IgA) builds up in the kidneys. This accumulation causes inflammation that can gradually damage the kidney’s filtering units, called glomeruli. Named after French physician Jean Berger who first described it in 1968, this condition is one of the most common types of glomerulonephritis worldwide.

The disease can develop at any age but most commonly appears in the late teens to late 30s. IgA nephropathy affects men more frequently than women and varies in severity from person to person. Some individuals may experience mild symptoms that remain stable for years, while others may progress to chronic kidney disease or even kidney failure. Early detection and proper management are crucial for preserving kidney function and preventing complications.

Recognizing the warning signs of IgA nephropathy is essential for timely medical intervention. While some people may have no noticeable symptoms initially, the following signs can indicate the presence of this kidney condition:

1. Blood in the Urine (Hematuria)

One of the most characteristic signs of IgA nephropathy is the presence of blood in the urine, medically known as hematuria. This is often the first symptom that brings patients to medical attention and can manifest in two different ways.

Visible blood (Gross hematuria): In some cases, the blood is visible to the naked eye, making the urine appear pink, red, or cola-colored. This type of hematuria often occurs during or shortly after an upper respiratory infection, such as a cold or flu. The appearance of dark or tea-colored urine following an illness is a hallmark feature of IgA nephropathy that distinguishes it from other kidney conditions. The visible blood typically appears within one to two days of the infection’s onset, which is notably quicker than in other types of kidney disease.

Microscopic hematuria: More commonly, the blood in the urine can only be detected through laboratory testing using a microscope or urine dipstick test. This microscopic hematuria may be discovered during routine medical examinations or health screenings when no symptoms are present. Many individuals with IgA nephropathy have persistent microscopic hematuria that remains undetected for years until a routine urinalysis reveals the abnormality.

The timing and pattern of hematuria can provide important clues about the condition. Episodes of visible blood in the urine may recur periodically, especially in connection with respiratory or other infections, while microscopic hematuria may persist continuously between episodes.

2. Foamy or Frothy Urine

The appearance of foamy or frothy urine is another significant warning sign of IgA nephropathy. This symptom occurs when excess protein leaks into the urine, a condition called proteinuria. When you urinate, the presence of protein creates bubbles that resemble the foam seen in beer or dish soap, and these bubbles may persist for several minutes rather than quickly disappearing.

Proteinuria occurs because the damaged glomeruli in the kidneys lose their ability to effectively filter waste products while retaining important proteins like albumin in the bloodstream. As the filtering mechanism becomes compromised, proteins that should remain in the blood pass through into the urine.

The amount of protein in the urine can vary considerably among individuals with IgA nephropathy. Some people may have only mild proteinuria that produces minimal foaming, while others develop nephrotic-range proteinuria, characterized by the loss of more than 3.5 grams of protein per day. Higher levels of protein loss are generally associated with more severe kidney damage and a greater risk of disease progression.

While occasional foamy urine can result from rapid urination or dehydration, persistent foamy urine—especially when accompanied by other symptoms—warrants medical evaluation. This symptom may worsen during periods of physical stress, illness, or increased disease activity.

3. Swelling (Edema)

Swelling, or edema, is a common symptom that develops in individuals with IgA nephropathy, particularly when significant amounts of protein are being lost in the urine. This fluid retention occurs because the kidneys are unable to properly regulate the body’s fluid and salt balance, and because low protein levels in the blood allow fluid to leak from blood vessels into surrounding tissues.

Common locations for swelling include:

• Feet and ankles: This is often the first area where swelling becomes noticeable, especially at the end of the day or after prolonged standing or sitting. You may notice that shoes feel tighter than usual or that socks leave indentations on the skin.
• Legs: As fluid retention progresses, swelling may extend upward to involve the lower legs and sometimes the thighs. The swelling may be more pronounced on one side or be symmetrical on both legs.
• Hands and arms: Some people notice puffiness in their hands, making it difficult to remove rings or causing a feeling of stiffness in the fingers, particularly in the morning.
• Face and eyes: Facial swelling, especially puffiness around the eyes (periorbital edema), is particularly noticeable in the morning upon waking. This occurs because fluid accumulates in facial tissues during sleep when lying flat.

The severity of edema can range from mild puffiness that’s barely noticeable to severe swelling that causes discomfort, limits mobility, and affects daily activities. Pressing on swollen areas may leave a temporary indentation in the skin, known as “pitting edema.” The extent of swelling often correlates with the severity of protein loss and kidney function impairment.

4. High Blood Pressure (Hypertension)

Elevated blood pressure is a frequent finding in people with IgA nephropathy and can be both a symptom and a complicating factor of the disease. Studies suggest that approximately 25-50% of individuals with IgA nephropathy develop hypertension at some point during their illness.

The kidneys play a crucial role in regulating blood pressure through several mechanisms, including controlling fluid volume and producing hormones that affect blood vessel constriction. When IgA nephropathy damages the kidneys, these regulatory mechanisms become impaired, leading to elevated blood pressure. The inflammation and scarring of kidney tissue can trigger increased production of renin, a hormone that raises blood pressure through a cascade of chemical reactions.

High blood pressure in IgA nephropathy often develops gradually and may not cause noticeable symptoms initially, which is why it’s sometimes called a “silent” problem. However, when blood pressure becomes significantly elevated, some individuals may experience:

• Headaches, particularly in the back of the head
• Dizziness or lightheadedness
• Blurred vision or other visual changes
• Shortness of breath
• Chest discomfort
• Nosebleeds

The relationship between high blood pressure and IgA nephropathy is bidirectional: the kidney disease causes hypertension, and uncontrolled hypertension further accelerates kidney damage, creating a vicious cycle. This makes blood pressure monitoring and management particularly important for preserving kidney function. Regular blood pressure checks are essential for anyone diagnosed with or suspected of having IgA nephropathy.

5. Flank or Back Pain

Some individuals with IgA nephropathy experience pain in the flank area (the sides of the body between the ribs and hips) or in the lower back region where the kidneys are located. This symptom, while not present in all cases, can be particularly noticeable during episodes of visible blood in the urine.

The pain associated with IgA nephropathy typically has distinct characteristics:

Nature of the pain: The discomfort is usually described as a dull ache rather than sharp or stabbing pain. It may feel like a persistent soreness or pressure in the kidney area. The intensity can range from mild to moderate and may be constant or intermittent.

Location: The pain is generally felt on one or both sides of the back, just below the rib cage. Because there are two kidneys, the pain may be bilateral (on both sides) or more prominent on one side if inflammation is more severe in one kidney.

Timing: Flank pain often coincides with episodes of acute inflammation, particularly when gross hematuria (visible blood in urine) appears following respiratory infections. During these flare-ups, the kidneys may become temporarily enlarged and inflamed, causing discomfort. The pain may subside as the acute episode resolves but can recur with subsequent flare-ups.

It’s important to note that kidney pain is different from common back pain caused by muscle strain or spinal issues. Kidney-related pain typically doesn’t change with body position or movement and isn’t relieved by rest or typical back pain remedies. If you experience persistent flank pain, especially when accompanied by other symptoms like blood in the urine or fever, medical evaluation is necessary to determine the cause.

6. Fatigue and Weakness

Persistent tiredness and a general sense of weakness are common complaints among people with IgA nephropathy, particularly as kidney function declines. This symptom can significantly impact quality of life but is often overlooked or attributed to other causes such as stress, poor sleep, or busy lifestyles.

The fatigue associated with IgA nephropathy has multiple underlying causes:

Anemia: As kidney function deteriorates, the kidneys produce less erythropoietin, a hormone that stimulates red blood cell production in the bone marrow. This leads to anemia, a condition characterized by insufficient red blood cells to carry adequate oxygen to the body’s tissues. The resulting oxygen deficit causes fatigue, weakness, and reduced stamina.

Waste product accumulation: Healthy kidneys filter waste products from the blood. When kidney function is impaired, toxins and metabolic waste products accumulate in the bloodstream, a condition called uremia. This buildup affects multiple body systems and contributes to feelings of tiredness and general malaise.

Inflammation: The chronic inflammatory process occurring in the kidneys can trigger systemic effects throughout the body, including fatigue. Inflammatory chemicals released during disease activity can affect energy levels and overall sense of well-being.

Nutritional factors: Protein loss through the urine can lead to nutritional deficiencies and muscle wasting, contributing to weakness and reduced physical capacity. Additionally, dietary restrictions sometimes recommended for kidney disease may inadvertently lead to insufficient calorie intake.

The fatigue may be mild and only noticeable during strenuous activities, or it can be severe enough to interfere with daily tasks and work. Many people describe feeling exhausted even after adequate sleep, lacking the energy to engage in activities they previously enjoyed, or needing frequent rest periods throughout the day. This symptom often develops gradually, and individuals may not realize how much their energy levels have declined until they look back at what they were able to do previously.

7. Decreased Urine Output and Changes in Urination Patterns

Changes in urination patterns can signal that IgA nephropathy is affecting kidney function. While not always present, especially in early stages of the disease, alterations in urine output and urinary habits can be important warning signs that warrant medical attention.

Reduced urine volume: As kidney function declines, the kidneys may produce less urine than normal, a condition called oliguria. Some individuals notice they’re urinating less frequently or producing smaller volumes with each bathroom visit. In more severe cases, urine output may decrease to dangerously low levels, indicating significant kidney function impairment.

Increased nighttime urination: Nocturia, or the need to wake up multiple times during the night to urinate, is common in people with kidney disease including IgA nephropathy. This occurs because damaged kidneys lose their ability to concentrate urine effectively, leading to increased urine production at night when the body would normally conserve fluid. Having to urinate two or more times per night regularly can disrupt sleep quality and contribute to daytime fatigue.

Changes in urine color and appearance: Beyond the blood in urine already mentioned, people with IgA nephropathy may notice other changes in urine appearance. The urine may appear darker or more concentrated, especially if urine output is reduced. Conversely, it may appear very pale or clear if the kidneys have lost concentrating ability. The persistent foamy appearance due to proteinuria may become more pronounced.

Urgency and frequency changes: Some individuals experience changes in urinary urgency (a sudden, strong need to urinate) or frequency (needing to urinate more often than usual). These symptoms may indicate that the disease is affecting bladder function or that secondary urinary tract issues have developed.

It’s helpful to pay attention to your normal urination patterns so you can recognize when changes occur. Tracking fluid intake and urine output over a few days can provide valuable information for healthcare providers in assessing kidney function. Any significant or persistent changes in urination should be discussed with a doctor, as they may indicate disease progression or the need for adjustment in management strategies.

Main Causes of IgA Nephropathy

The exact cause of IgA nephropathy remains not fully understood, but researchers have identified several factors that contribute to its development. The condition involves a complex interplay of immune system abnormalities, genetic factors, and environmental triggers.

Abnormal IgA antibody production: The primary problem in IgA nephropathy involves the production of abnormal immunoglobulin A (IgA) antibodies. In healthy individuals, IgA antibodies help protect against infections, particularly in the mucous membranes of the respiratory and digestive tracts. In people with IgA nephropathy, the body produces IgA molecules with abnormal sugar molecules attached to them (galactose-deficient IgA1). These abnormal antibodies are recognized as foreign by the immune system, which creates additional antibodies against them, forming immune complexes that deposit in the kidneys and cause inflammation.

Genetic predisposition: Research indicates that genetic factors play a role in IgA nephropathy. The disease tends to run in families, and certain genetic variations have been associated with increased risk. Multiple genes involved in immune function and IgA production have been implicated, though no single gene causes the condition. People of Asian and Caucasian descent appear to be at higher risk than those of African descent.

Immune system dysfunction: Beyond the production of abnormal IgA, other aspects of immune system function are impaired in IgA nephropathy. The body’s mechanisms for clearing abnormal IgA from the bloodstream may be deficient, allowing these molecules to accumulate and deposit in the kidneys. Additionally, once immune complexes form in the kidneys, the inflammatory response they trigger may be excessive or poorly regulated.

Infectious triggers: Respiratory, urinary, and gastrointestinal infections often precede or coincide with disease flare-ups, suggesting that infections may trigger increased production of abnormal IgA. This connection is particularly evident in episodes of visible blood in the urine that occur shortly after respiratory infections. However, infections are thought to trigger rather than cause the underlying disease process.

Environmental and lifestyle factors: While not direct causes, certain factors may influence disease expression or progression. These include dietary factors, particularly gluten in individuals with celiac disease or gluten sensitivity, and possibly other environmental exposures. However, the relationship between these factors and IgA nephropathy is still being researched.

Prevention Strategies

Because the exact cause of IgA nephropathy is not fully understood and involves genetic factors that cannot be changed, there is no certain way to prevent the disease from developing. However, for individuals already diagnosed with IgA nephropathy, several strategies can help slow disease progression and prevent complications:

Regular medical monitoring: Consistent follow-up with a healthcare provider or nephrologist (kidney specialist) is essential. Regular monitoring of kidney function through blood tests (creatinine, estimated glomerular filtration rate) and urine tests (protein levels, hematuria) allows for early detection of disease progression and timely adjustment of management strategies.

Blood pressure control: Maintaining optimal blood pressure is one of the most important factors in protecting kidney function. Regular blood pressure monitoring at home and adherence to medical recommendations for blood pressure management can significantly slow kidney damage progression.

Dietary modifications: While specific dietary recommendations should be individualized based on disease stage and severity, some general principles may help:

• Limiting sodium (salt) intake to help control blood pressure and reduce fluid retention
• Moderating protein intake as recommended by healthcare providers, as excessive protein may increase kidney workload
• Maintaining adequate but not excessive fluid intake
• For individuals with celiac disease or gluten sensitivity, adhering to a gluten-free diet may be beneficial

Healthy lifestyle habits: General health measures that support overall wellbeing and kidney health include:

• Maintaining a healthy body weight
• Engaging in regular physical activity as tolerated
• Avoiding smoking and excessive alcohol consumption
• Managing stress through appropriate techniques
• Getting adequate sleep

Infection management: Since infections can trigger disease flare-ups, taking steps to prevent and promptly treat infections may be helpful. This includes practicing good hygiene, staying up to date with vaccinations, and seeking medical attention early when infections occur.

Avoiding nephrotoxic substances: Certain medications and substances can harm the kidneys. People with IgA nephropathy should consult healthcare providers before taking new medications, including over-the-counter pain relievers (particularly nonsteroidal anti-inflammatory drugs like ibuprofen), and should avoid exposure to known kidney toxins.

Managing coexisting conditions: Effectively managing other health conditions such as diabetes, high cholesterol, and obesity can help protect kidney function and overall health.

While these measures cannot prevent IgA nephropathy from initially developing, they represent an important approach to preventing progression and maintaining kidney function for as long as possible once the disease is diagnosed.

Frequently Asked Questions

Is IgA nephropathy the same as Berger’s disease?

Yes, IgA nephropathy and Berger’s disease are the same condition. It is called Berger’s disease after Dr. Jean Berger, the French physician who first identified and described the condition in 1968. Both terms are used interchangeably in medical literature and practice.

Can IgA nephropathy go away on its own?

IgA nephropathy is generally considered a chronic, lifelong condition that does not spontaneously resolve. However, the disease course varies greatly among individuals. Some people experience very slow or no progression for many years or even decades, while others progress more rapidly. Complete spontaneous remission is rare but has been documented in some cases.

How is IgA nephropathy diagnosed?

IgA nephropathy is definitively diagnosed through a kidney biopsy, a procedure in which a small sample of kidney tissue is removed and examined under a microscope. The biopsy reveals characteristic deposits of IgA antibodies in the glomeruli. Prior to biopsy, blood and urine tests showing hematuria and proteinuria may suggest the diagnosis, but confirmation requires tissue examination.

Does everyone with IgA nephropathy develop kidney failure?

No, not everyone with IgA nephropathy will develop kidney failure. The disease course is highly variable. Studies suggest that approximately 20-40% of people with IgA nephropathy may progress to end-stage kidney disease requiring dialysis or transplantation within 20 years of diagnosis. However, many individuals maintain stable kidney function or experience only slow decline with proper management. Factors such as the amount of proteinuria, blood pressure control, and degree of kidney damage at diagnosis influence prognosis.

Can infections make IgA nephropathy worse?

Yes, infections, particularly upper respiratory infections, can trigger flare-ups of IgA nephropathy. Many people with the condition notice that their urine becomes visibly bloody during or shortly after colds, flu, or other infections. These episodes represent increased disease activity and inflammation in the kidneys. While individual infection episodes are usually temporary, repeated or severe flares may contribute to progressive kidney damage over time.

Should I follow a special diet if I have IgA nephropathy?

Dietary recommendations for IgA nephropathy should be individualized based on your specific situation, kidney function level, blood pressure, and other factors. Generally, limiting sodium intake is recommended to help control blood pressure and reduce fluid retention. Some healthcare providers may recommend moderating protein intake, especially if you have significant proteinuria. If you have celiac disease or gluten sensitivity, a gluten-free diet may be beneficial. Always consult with your healthcare provider or a registered dietitian familiar with kidney disease before making significant dietary changes.

Can IgA nephropathy affect children?

Yes, IgA nephropathy can occur in children, though it is more commonly diagnosed in adults. When it does affect children, it is often discovered during routine urine tests or after an episode of visible blood in the urine following an infection. The disease course in children may differ somewhat from adults, and some studies suggest that children may have better long-term outcomes, though this is not universal.

When should I see a doctor about possible IgA nephropathy symptoms?

You should seek medical attention if you notice blood in your urine (especially if it appears after an infection), persistent foamy urine, unexplained swelling, or if you have risk factors for kidney disease. Anyone with a family history of IgA nephropathy or kidney disease should be particularly vigilant about these symptoms. Early detection and management can help preserve kidney function and prevent complications, so don’t hesitate to discuss concerning symptoms with your healthcare provider.

References:

• National Kidney Foundation – IgA Nephropathy
• Mayo Clinic – IgA Nephropathy (Berger’s Disease)
• National Institute of Diabetes and Digestive and Kidney Diseases – IgA Nephropathy
• Kidney Health Australia – IgA Nephropathy
• UpToDate – IgA Nephropathy: Beyond the Basics