IgA nephropathy, also called Berger’s disease, is a kidney condition where a protein called immunoglobulin A (IgA) accumulates in the kidneys. This leads to inflammation that can eventually impair the kidney’s ability to filter waste from the blood.
Progression of IgA nephropathy typically occurs gradually over time, with varying severity among individuals. While some may only experience blood or protein in their urine, others may develop complications like kidney function loss, hypertension, and lower extremity edema. In severe cases, kidney failure can occur, resulting in insufficient waste filtration by the kidneys.
IgA nephropathy cannot be cured, but medications can help slow it down. Treatment may be necessary to reduce inflammation, decrease protein in the urine, and prevent kidney failure. Managing blood pressure and cholesterol levels is also needed to prevent complications.
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IgA Nephropathy FAQ’s
Think of your kidneys as small but powerful filtration plants whose job is to keep your blood clean and the body’s chemical balance maintained. Each day the kidneys process about 200 quarts of fluid through their two million tiny treatment plans, the nephrons.
Within the nephron is the glomerulus, a tangle of fine capillaries that filter the blood before passing it on to the tubules, where the kidneys continually adjust the filtrate to your body’s needs, adding back chemicals removed during filtration or drawing off more water. What’s needed by the body is returned to the bloodstream; what’s not needed is excreted as urine.
Diagnosing IgA nephropathy typically involves a detailed medical history, physical exam, blood and urine tests, imaging studies, and confirmation of IgA deposits in the kidneys via kidney biopsy.
This comprehensive approach helps healthcare providers accurately diagnose and formulate a treatment plan for patients with this condition.
Tests to Find IgA Nephropathy
Several tests may be used to get the complete picture of your kidney health and see whether you have IgAN:
- Urinalysis: a general urine (pee) test that looks for blood and/or protein in your urine.
- Urine protein-creatinine ratio (uPCR): a urine test that measures the level of protein and creatinine (waste product) in your urine. This test is very similar to the urine albumin-creatinine ratio (uACR) but measures all the different proteins that may be present (not just albumin).
- Estimated glomerular filtration rate (eGFR): a blood test that helps assess how well your kidneys are removing waste products from the blood.
- Kidney biopsy: a test in which one or more tiny pieces (samples) of your kidney is removed and then looked at with a microscope. These results can also help predict your long-term risk for kidney failure.
There is no current cure for IgAN and that is why the IgA Nephropathy Foundation was founded. Since the disease varies from one person to another, there is no definite progression or course that the disease will take.
What works for one person may have virtually no effect on another, but our goal is to bring those with IgAN together to share experiences and bring awareness to this disease.
Since there is no cure, treatments focus on slowing the progression of the disease and preventing complications.
Some individuals experience complete remission, while others live normal lives with medications to treat their symptoms (such as high blood pressure meds, immunosuppressants, omega 3 fatty acids & vitamin E supplements), yet others experience complete kidney failure (end-stage kidney disease) and require either dialysis or a kidney transplant depending on the severity. It is estimated that as many as half of those affected with IgAN will develop end-stage renal disease. This poses one large problem… there aren’t enough kidneys and many patients wait on the transplant list for years.
IgA nephropathy, or Berger’s disease, is the most common type of glomerulonephritis, a form of kidney inflammation, worldwide. The prevalence of IgA nephropathy varies significantly by region, with the highest rates reported in East Asia and the lowest in North America.
While exact numbers can be difficult to determine due to underdiagnosis and varying medical practices, it is estimated that IgA nephropathy affects roughly 1 in 100,000 people in the United States and Europe. In regions like Japan and China, the prevalence is higher, with estimates suggesting it could be as common as 1 in 10,000 people. The condition often remains undiagnosed until kidney function is significantly impaired, making these estimates potentially lower than the actual number of cases.
IgA Nephropathy is not a hereditary disease, like cystic fibrosis or Huntington’s chorea. In certain families, there does appear to be a predisposition to develop the disease, suggesting a genetic component. But before you can develop IgA Nephropathy, there must be some kind of trigger. We just don’t know what that is.If you are worried about your children possibly developing IgA Nephropathy, make sure to have their urine tested as part of a routine physical exam. Urinary abnormalities are fairly common, so don’t assume the child has IgAN if a single test shows a problem. That’s just a signal that more thorough testing is necessary.
Occasionally there are families with two or more cases of IgAN, but in our experience this has generally been sex-linked (e.g., mother-daughter, uncle-nephew).
IgAN was thought relatively benign when it was first identified in 1968 by the French doctor, Jean Berger, but gradually researchers realized that in up to 50% of the cases, the disease very slowly progresses to end-stage renal failure, requiring major lifestyle changes, dialysis, and possibly transplantation.
IgAN does not immediately interfere with the kidneys’ filtration work — except in acute cases — but it may threaten it ultimately. Damage results as kidney tissue gradually develops irreversible scarring, which ultimately blocks renal capillaries, causing the death of surrounding tissues as these are deprived of blood and nutrients. The slow accumulation of these tiny affected areas can take ten or twenty — or more — years to produce kidney failure, although a small minority of patients, mainly adults, have a rapidly progressive form that results in renal failure within a few years or even months.
As individual capillaries become clogged by coagulation excessive cell growth, or infiltration by inflammatory cells, the nephron loses filtering surface. The kidneys’ glomerular filtration rate [GFR], a measure of how efficiently the kidneys filter blood, declines when that loss is enough to overcome the body’s efforts to adapt by increasing pressure throughout the remaining nephrons. As GFR decreases, the kidneys nose their ability to cleanse the blood of toxins and metabolic byproducts. Kidney failure and uremic poisoning result.
It is difficult to predict from initial clinical signs and biopsy results just which patients will have which course. Statistical analyses have yielding conflicting results, but some factors do seem linked to a poor prognosis. Unremittingly heavy proteinuria, uncontrolled hypertension, declining glomerular filtration rate, high serum creatinine, and being age 30 or older at the time of apparent onset of the disease are some of the factors tied to a poor prognosis. Severe lesions in the initial biopsy, especially evidence of sclerosis [scarring] or crescent formation are also ominous signs.
Except for the very few who have rapidly progressive IgAN, most patients will take years, even decades, to develop kidney failure. Those who have end-stage renal disease [ESRD] are treated with dialysis and may be candidates for transplantation. Deposits of IgA, the hallmark of the disease, frequently appear in transplanted kidneys, but the clinical symptoms do not usually come back. An early survey of transplants due to IgAN showed that although IgAN-type lesions recurred in over 50% of the respondents, only 3% lost their new kidneys to a resurgence of the disease.
More recently, doctors have observed deposits of immunoglobulin A in up to 80% of patients who have received transplants after their own kidneys were destroyed by IgAN; yet only one quarter of these showed any clinical symptoms. About 20% of those transplanted lose their grafts to recurrent IgAN. According to one nephrologist who has followed this subject, graft failure came generally after 8-10 years, which would be the expected lifespan for a cadaver kidney anyway. In other words, recurrent IgAN did not contribute to early, unexpected loss of a transplant.
The possibility of recurrent IgAN should not discourage patients from having a transplant, but they should be aware that a transplant is not a cure for the disease, merely a replacement organ.
Your doctor may want to put you on a low-salt diet to minimize stress of the kidneys and prevent excess fluid retention that would strain your heart. This is especially likely if you have shown signs of edema or hypertension. Even if you are not showing such signs, a low-sodium diet may be a good idea. It won’t hurt and it may well help, particularly for those being treated with prednisone, which tens to make the body retain sodium.
How strict a diet is the question? When we think “low sodium,” we think of eliminating potato chips, pretzels, cocktail peanuts — all snack foods with added table salt (sodium chloride). But sodium appears in other forms, such as sodium bicarbonate (baking soda), sodium propionate (a preservative), dihydroxyaluminum sodium carbonate (antacids), sodium fluoride (toothpaste), and monosodium glutamate (m.s.g.). Whether the culprit is all forms of sodium or only table salt is not clear. Sodium chloride is naturally high in some foods, from soup to pickles to breakfast cereals. Even some frozen vegetables, such as peas and lima beans, have added salt.
Whether your low-salt diet is very strict (no more than 1,000 mg of sodium per day), or more lenient (2,000 mg per day), you will probably have to eliminate certain favorites, namely, all fast foods, pizza, cheesesteaks, hoagies, prepared snacks, etc. Plan on becoming an avid label-reader. Health food stores and most supermarkets offer low-salt or no-salt substitutes for some items (catsup, mayonnaise, deli meats and cheeses, salad dressings, crackers, cookies, potato and taco chips, soups, candies, etc.). They also offer assortments of herbs that can be used in lieu of salt (not to be confused with commercial salt substitutes that rely on potassium in place of sodium; these are not recommended).
You may find it easier to put the entire family on a low-salt regime, so the patient doesn’t feel “left out.” It’s healthier for everyone, but it does take cooperation on the family’s part and flexibility on the cook’s. Paradoxically, the new diet may be more easily accepted if you radically revamp your cooking style rather than trying to remake old favorites or introduce low-sodium analogs to familiar foods. Ethnic cuisines that rely heavily on herbs or citrus juices — for example, Italian, Mexican, and Thai — are more easily adapted to low-salt cooking.
Another possibility is to adopt a basically vegetarian diet. Those with moderate to heavy proteinuria should strongly consider eliminating meat and milk products, basing their diet upon fresh fruits and vegetables, whole grains, and such vegetable sources of protein as nuts, beans, legumes, soy, and fish, while avoiding processed or fast foods. This type of diet saves the kidneys a lot of hard work in clearing the body of the byproducts of metabolizing animal proteins. There are also chemicals in milk and meat that exacerbate the inflammatory process taking place in the kidneys.
IgA nephropathy, also known as Berger’s disease, is believed to have a genetic component, but it is not strictly classified as a genetic condition. Research has shown that IgA nephropathy can run in families, suggesting that certain genetic factors may predispose individuals to the disease. However, the exact genetic mechanisms are not fully understood, and environmental factors also play a role in the development and progression of the disease. The interplay between genetics and other factors like infections, immune responses, and lifestyle can influence the onset and severity of IgA nephropathy.
Key Points About IgA Nephropathy and Genetics:
Research Ongoing: Studies continue to explore the genetic basis of IgA nephropathy to better understand its causes and potential treatments.
Family History: IgA nephropathy often occurs in families, indicating a potential hereditary risk.
Genetic Predisposition: Certain genetic variations have been linked to an increased risk of developing IgA nephropathy.
Not Solely Genetic: Environmental factors, such as infections or stress, also contribute to the development of the disease.
Complex Inheritance: The inheritance pattern of IgA nephropathy is complex and not fully understood.
Can IgA nephropathy lead to kidney failure?
In certain instances, IgA nephropathy, a kidney disease characterized by inflammation of the glomeruli, can advance to end-stage renal disease (renal failure) if not adequately addressed. It is imperative for individuals with this condition to undergo routine monitoring and receive timely medical care to avert the risk of kidney failure.
Early detection and intervention play a pivotal role in managing IgA nephropathy and preventing further kidney harm.
What is the prognosis for individuals with IgA nephropathy?
The prognosis for individuals with IgA nephropathy varies depending on the severity of the condition, kidney biopsy findings, the amount of protein in the urine, response to treatment, and overall kidney function. With proper management and medical care, many individuals with IgA nephropathy can lead a relatively normal life.
Making lifestyle changes is essential for managing IgA nephropathy and supporting kidney health. This includes following a diet that is low in salt and protein and maintaining a healthy weight. Regular exercise, avoiding smoking, and limiting alcohol consumption are also important.
Incorporating these habits into your daily routine can help improve your overall health and potentially slow the progression of IgA nephropathy. It is crucial to prioritize these lifestyle changes in order to manage the condition and reduce the risk of complications effectively.
What is Immunoglobulin A (IgA)?
Immunoglobulin A (IgA) is one of the five major types of antibodies produced by the immune system to protect the body from infections. It plays a crucial role in defending mucosal surfaces, such as those in the respiratory and gastrointestinal tracts, by binding to harmful pathogens like bacteria and viruses, preventing them from entering cells and causing illness. Immunoglobulin A is primarily found in mucous membranes, saliva, tears, and breast milk, making it a first line of defense against pathogens in areas exposed to the external environment. Its protective role extends to helping maintain a balanced immune response and guarding against infections.
Key Functions of IgA
- Helps in neutralizing toxins produced by bacteria and viruses.
- Protects mucous membranes in the respiratory and digestive systems.
- Prevents pathogens from attaching to and invading epithelial cells.
- Found in body fluids such as saliva, tears, and breast milk.
- Plays a role in allergic responses and autoimmune conditions.
IgA Nephropathy Symptoms
IgA nephropathy may be asymptomatic initially. Symptoms may not appear for over a decade. Occasionally, the presence of protein and red blood cells in the urine, visible under a microscope, is discovered through routine medical tests.
When IgA nephropathy causes signs and symptoms, they might include:
- Urine can appear cola- or tea-colored due to blood. This can occur following a cold, sore throat, or respiratory infection.
- Blood that can be seen in the urine.
- Proteinuria is when protein leaks into the urine, causing it to appear foamy.
- Discomfort in the lower back on either side beneath the ribs.
- Edema is the medical term for swelling in the hands and feet.
- High blood pressure.
- Weakness and tiredness.
If the disease leads to kidney failure, symptoms may include:
- Rashes and itchy skin.
- Muscle cramps.
- Upset stomach and vomiting.
- Less appetite.
- Metallic taste in the mouth.
- Confusion.
Kidney failure is fatal if untreated, but dialysis or a kidney transplant can prolong life significantly.
IgA Nephropathy Causes
When blood enters the glomerulus, only small molecules like water, minerals, and waste pass through the capillary walls, while large molecules like proteins and red blood cells do not. The filtered part moves to the tubule in the nephron, where water, nutrients, and minerals are returned to the bloodstream, and excess water and waste become urine that goes to the bladder.
The kidneys are kidney-shaped organs located at the back, one on each side of the spine. They contain glomeruli that filter waste from the blood. The filtered blood returns to circulation, while waste is eliminated in urine.
IgA is an antibody protein produced by the immune system to combat germs and infections. In IgA nephropathy, this protein accumulates in the glomeruli, leading to inflammation and decreased filtering function.
Elevated levels of galactose-deficient IgA are associated with certain autoimmune conditions, particularly IgA nephropathy. In this condition, the immune system produces an abnormal form of IgA that is deficient in galactose. This galactose-deficient IgA can form immune complexes that deposit in the kidneys, leading to inflammation and potential kidney damage. Understanding the role of galactose-deficient IgA in these processes is crucial for developing targeted therapies and improving outcomes for affected individuals.
Researchers don’t know exactly what causes IgA to build up in the kidneys. But the following things might be linked with it:
- Genes: IgA nephropathy is more common in some families and in certain ethnic groups, such as people of Asian and European descent.
- Liver diseases: These include scarring of the liver called cirrhosis and chronic hepatitis B and C infections.
- Celiac disease: Eating gluten, a protein found in most grains, triggers this digestive condition.
- Infections: These include HIV and some bacterial infections.
IgA Disease Risk factors
The exact cause of IgA nephropathy is unknown. However, factors like gender, ethnicity, age, and family history may increase the risk of developing the condition. Men in North America and Western Europe are affected more than women. White people and those of Asian descent are more likely to have IgA nephropathy compared to Black individuals. The condition typically occurs in individuals between mid-teens and mid-30s and may run in families.
- Sex: In North America and Western Europe, IgA nephropathy is more common in men than in women, with at least double the number of affected men.
- Ethnicity: IgA nephropathy is more prevalent in Caucasian and Asian individuals compared to African Americans.
- Age: IgA nephropathy typically starts from teens to late 30s. .
- Family history: IgA nephropathy seems to be hereditary in certain families.
IgA Nephropathy Complications
IgA nephropathy progresses differently in each person. Some have mild symptoms for years, while others go undiagnosed. Complications may arise in some individuals.
- High blood pressure: IgA buildup in the kidneys can lead to increased blood pressure, which in turn can harm the kidneys further.
- High cholesterol: Elevated cholesterol levels increase the likelihood of experiencing a heart attack.
- Acute kidney failure: When IgA buildup impairs kidney filtration, blood waste levels rise can cause acute kidney injury and it some rare cases leading to rapidly progressive glomerulonephritis.
- Chronic kidney disease: IgA nephropathy can lead to kidney failure, necessitating dialysis or a kidney transplant for survival.
- Nephrotic syndrome: This collection of issues stems from glomeruli damage and can result in elevated urine protein, decreased blood protein, lipid levels, and swelling in the eyelids, feet, and abdomen.
IgA Nephropathy Prevention
Unfortunately, IgA nephropathy is a condition that cannot be prevented. It is important to speak with your doctor, especially if there is a family history of kidney problems.
By discussing ways to maintain kidney health, such as managing high blood pressure and cholesterol levels, you can potentially slow down the progression of the disease and improve your overall quality of life. Regular monitoring and following a healthy lifestyle are key to effectively managing IgA nephropathy.
When to see a doctor if you have IgA Disease
If you suspect you have IgA nephropathy symptoms, consult your doctor. If you see blood in your urine, it’s important to get evaluated. Persistent or unexplained occurrences could indicate a serious health issue. Seek medical attention for sudden swelling in your hands or feet.
References
- IgA nephropathy. National Kidney Foundation.https://www.kidney.org/atoz/content/iganeph.
- Kidney failure (ESRD) – Symptoms, causes and treatment options. American Kidney Fund. https://www.kidneyfund.org/all-about-kidneys/kidney-failure-symptoms-and-causes
- IgA Nephropathy (Berger Disease). https://www.ncbi.nlm.nih.gov/books/NBK538214/
- Immunoglobulin A (IgA) Nephropathy. https://www.hopkinsmedicine.org/health/conditions-and-diseases/immunoglobulin-a-iga-nephropathy
- IgA nephropathy. American Kidney Fund. https://www.kidneyfund.org/all-about-kidneys/other-kidney-diseases/iga-nephropathy/
- Hematuria. Urology Care Foundation. https://www.urologyhealth.org/urology-a-z/h/hematuria
- What is IgA Nephropathy (Berger’s Disease)?. https://www.pennmedicine.org/for-patients-and-visitors/patient-information/conditions-treated-a-to-z/bergers-disease-iga-nephropathy
- Wyatt RJ, Julian BA. IgA nephropathy. New England Journal of Medicine. 2013;368(25):2402–2414.
- IgA nephropathy. MedlinePlus website. www.nlm.nih.gov/medlineplus/ency/article/000466.htm . Updated February 26, 2014. Accessed October 19, 2015.
- Brake M. IgA nephropathy. Medscape website. emedicine.medscape.com/article/239927-overview . Updated April 6, 2015. Accessed October 19, 2015.
- Satpathy HK. IgA nephropathy. In: Ferri FF, ed. Ferri’s Clinical Advisor 2013. 1st ed. St. Louis: Mosby; 2012: 570–571.
- IgA nephropathy. National Kidney Foundation website. www.kidney.org/atoz/content/iganeph . Accessed October 19, 2015.