What Is Autoimmune Kidney Disease? (2023)

  • Overview
  • What is IgA nephropathy?
  • How common is IgA nephropathy?
  • Who is at risk for IgA nephropathy?
  • How does IgA nephropathy affect the kidneys?
  • What are the symptoms of IgA nephropathy?
  • What causes IgA nephropathy?
  • Diagnosis
  • Treatment of IgA nephropathy
  • Managing IgA nephropathy
  • Complications associated with IgA nephropathy
  • When to see a doctor
  • The lowdown
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What is IgA nephropathy?

IgA nephropathy¹ happens when your immune system attacks your kidneys. It is also known as autoimmune kidney disease and Berger's disease. It is considered an autoimmune disease that affects the kidneys and can run in families, although often there is no family history. The damage done can lead to chronic kidney disease and eventually to kidney failure.

It is the third most common cause of kidney disease, although not as common as diabetic kidney disease. It is called IgA nephropathy because a key part of the disease is your normal IgA antibodies building up excessively in your kidneys.

How common is IgA nephropathy?

Again, IgA nephropathy is the third most common cause of kidney disease. However, it is still considered a rare disease. About 60,000 people² in the US have the disease at any given time.

Who is at risk for IgA nephropathy?

Most patients are diagnosed in their teens through their late thirties. Those most at risk are men, people of Asian or Caucasian ethnicity, and members of the Zuni and Navajo tribes.

Again, the disease is considered rare, and risk factors are as yet poorly understood.

How does IgA nephropathy affect the kidneys?

IgA nephropathy happens when you produce IgA antibodies that have insufficient amounts of a sugar called galactose. This sugar is part of the mechanism which allows your white blood cells to recognize each other.

(Video) Immunologic Therapy at Ohio State for Autoimmune Kidney Disease

Because of this, other circulating antibodies attach themselves to the faulty IgA, forming a clump called an immune complex. These clumps become stuck in the glomeruli, the small looping blood vessels inside your nephrons, which are the part of the kidney that actually filters your blood. This, in turn, causes high levels of inflammation and damage.

Your kidneys will leak blood and protein into the urine, and the damage done will cause the slow development of scarring of the nephrons. Eventually, this can lead to kidney failure and the need for a transplant or dialysis.

What are the symptoms of IgA nephropathy?

Many people with IgA nephropathy have had no symptoms for years, and some people may have it and not even know it. The first symptoms, when and if they do occur, include:

  • Blood in the urine, presenting as either pink or dark brown “tea-colored”, which may occur after a cold, sore throat, or respiratory infection.

  • Excessively foamy urine - an indication of excessive protein loss. Often it may be described as the kind of foam you get when you shake a bottle of soft drink.

  • Weakness and fatigue

  • Pain in the lower back or sides

  • Swelling in your hands or feet

  • High blood pressure

IgA nephropathy is sometimes spotted when a urine test is done to check for another disease. The doctor may find blood or protein in your urine and decide to investigate the possibility of kidney damage.

What causes IgA nephropathy?

The cause of IgA nephropathy is poorly understood. We do not yet know why some people produce galactose-deficient IgA antibodies. Because the disease is so rare, it is harder to do the kind of analysis needed to establish the root cause. There may be some genetic links. It may also occur as part of your immune system's response to respiratory or intestinal infections.

Risk factors

There are several risk factors for IgA nephropathy. Most patients are diagnosed in their teens through their late thirties. Risk factors include:

  • Male sex

  • Asian or Caucasian race

  • A family history of either IgA nephropathy or IgA vasculitis³ (The latter condition causes IgA to collect in small blood vessels throughout the body.)

  • Celiac disease

    (Video) IgA nephropathy - causes, symptoms, diagnosis, treatment, pathology

  • HIV infection

  • Cirrhosis of the liver

  • Being a member of the Zuni or Navajo tribe

While there may be some genetic predisposition, it’s thought that a second hit to the immune system is needed before the disease is triggered (e.g., by an infection stimulating your immune system).


Kidney disease, in general, is most often diagnosed by detecting blood or protein in the urine. The latter is called albuminuria and means your urine has an unusual amount of albumin, which is a protein typically found in the blood.

Your kidneys typically prevent blood and protein from leaking into the urine, but damage to the glomeruli can prevent this filtering process from working correctly. This means that kidney disease may be found during a routine urine test.

Diagnosis is typically done using urine and blood tests and a medical and family history. Urine tests are done to look for albumin or blood in the urine. Meanwhile, a blood test may be done to estimate your glomerular filtration rate (eGFR), which is a way to estimate how much blood your kidneys are filtering per minute and, thus, how efficiently they are working.

These tests can diagnose kidney disease, but confirming IgA nephropathy requires a kidney biopsy and the examination of tissue under a microscope.

If your doctor orders a biopsy, you will get it done in a hospital or clinic under local anesthesia and light sedation. A small needle will be inserted into the kidney to obtain the sample.Only a biopsy can confirm the presence of IgA deposits in your glomeruli.

Treatment of IgA nephropathy

There is no specific treatment for IgA nephropathy, and scarring from it cannot be repaired. However, many cases resolve themselves before too much damage is done.

If this is not the case, then treatment focuses on slowing the progression of the disease to avoid progression to end-stage kidney disease (kidney failure).

Treatments include:

Antihypertensive medication

IgA nephropathy can cause high blood pressure, so you may be prescribed one of two antihypertensive medications that have also been shown to slow the progress of kidney disease. These are either angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs). These tablets help reduce blood pressure and protein leakage and may therefore reduce the chance of permanent kidney damage.

If your blood pressure is still high, you may be given other medications such as calcium channel blockers or beta blockers.


You may be prescribed a diuretic to help your kidneys remove extra fluid from the blood. Kidney problems cause high blood pressure by reducing the efficiency of your kidneys when they filter blood and generate urine, and a diuretic can help compensate for this.


These are a relatively new class of medications that can also help reduce protein leakage from the kidney. They have a mild diuretic and blood pressure-lowering effect as well.

(Video) What are autoimmune disorders?

Other medications

You may be given other medications. These include corticosteroids or cyclophosphamide to reduce inflammation and help "dial down" your immune system or statins to keep cholesterol levels down (IgA nephropathy can elevate cholesterol).

If you are on corticosteroids or cyclophosphamide, these drugs can suppress your immune system, which can make you more vulnerable to infections.

A recently-approved drug that is sometimes used is budesonide.⁴ Budesonide reduces the amount of protein excreted in your urine but is still being studied to establish its impact on disease progression. Budesonide is also an immunosuppressant drug.

However, one issue is that the drug can raise blood pressure, which may cause issues for many patients.

Dietary changes

There is no indication that diet and nutrition play any role in causing NgA nephropathy. However, you may be recommended certain dietary changes to support renal health in general. Typically this means limiting sodium and reducing saturated fat and cholesterol.

Reducing cholesterol is particularly important as IgA nephropathy can cause cholesterol levels to rise.


While it is still under investigation, studies show that tonsillectomy⁵ may slow disease progression. This might seem like a strange connection, but removing the tonsils apparently lowers the amount of galactose-deficient IgA1 produced. The procedure is relatively safe, but again, more research is needed to establish whether this is a good treatment and which patients it might benefit.

Fish oil

There are some indications that taking fish oil may slow the rate of loss of renal function. This appears to be the result of the anti-inflammatory properties of omega-3 fatty acids. Fish oil generally has no adverse effects and has other health benefits.

However, you should still talk to your doctor before taking it, and you should not use it instead of medication unless specifically recommended.

Some experts believe that the right dosage of omega-3 fatty acids may allow for the reduced use of corticosteroids and, thus, a reduction in immunosuppressant effects. However, more research still needs to be done.

Managing IgA nephropathy

As already mentioned, your doctor may recommend you reduce dietary sodium, fat, and cholesterol. Most people with IgA nephropathy can lead a normal life for many years, although eventually (typically after 20 to 25 years), there is a risk of kidney failure.

If you are on immunosuppressant drugs, you should attempt to avoid active infection, not be around people who are sick, and consider wearing a well-fitting protective mask if there is something "going around."

Some people experience chronic fatigue that can interfere with everyday activities. Others may have no symptoms at all other than episodes of blood in the urine.

The disease progresses slowly in most cases, and many people never experience kidney failure.

Your doctor may recommend you monitor your blood pressure at home. Many people benefit from joining a support group in their area.

If your disease progresses to end-stage kidney disease, you may need to go on dialysis. Follow the instructions of your care team, especially limiting fluid consumption. Some people with IgA nephropathy are candidates for a kidney transplant if a compatible organ can be found.

You should have your urine tested regularly to check the progression of the disease. Some people may find it goes into complete remission and that they can stop taking medication, although it is a good idea to continue with regular screening, just in case.

(Video) Connect, Learn and Manage IgA Nephropathy

Pregnancy can cause significant complications for people with IgA nephropathy, potentially resulting in renal insufficiency that can cause poor pregnancy outcomes and worsen your prognosis. However, if you have a good renal function and low blood pressure, you can have a successful pregnancy.

Talk to your doctor if you are attempting to conceive, and make sure that you have a care team experienced in managing pregnancy in people with chronic kidney disease. Your medications may need to be changed once you’re pregnant as they may cause harm to the fetus.

Complications associated with IgA nephropathy

IgA nephropathy can lead to both acute and chronic kidney failure. It can also cause various other complications, including:

  • High blood pressure

  • Nephrotic syndrome. This is a collection of symptoms indicating kidney damage, which include elevated protein in your urine, reduced protein in your blood, high cholesterol, and fluid retention.

  • Cardiovascular problems

  • Henoch-Schönlein purpura,⁶ also called IgA vasculitis. This means the small blood vessels in your skin, joints, intestines, and kidneys become inflamed and bleed. The key sign is red or purple spots on the skin. But it can also cause symptoms including headache, fever, loss of appetite, cramping and abdominal pain, joint pain, hives, bloody diarrhea, and painful menstruation.

The link between IgA nephropathy and IgA vasculitis is particularly strong, and it appears some of the same genes are involved in both. Taking steps to slow the progression of your disease can help slow these symptoms.

Most people with IgA nephropathy will need medication to treat high blood pressure.

When to see a doctor

You should always see a doctor if you have blood in your urine or if your urine is discolored. However, be aware that blood in your urine is more likely a sign of something much more common and less serious, such as a urinary tract infection or a kidney stone.

Many cases of IgA nephropathy are discovered while your doctor is looking for something else. However, if you have bloody or foaming urine and are also experiencing unusual fatigue, kidney disease of some kind should be suspected.

People with IgA nephropathy should see their doctor regularly to have tests to monitor their kidney function.

The lowdown

Autoimmune kidney disease or IgA nephropathy is a rare condition where a deficiency in your immune system, specifically in the production of certain antibodies, can cause damage to your kidneys. This condition has no specific treatment, although significant research is being done. Fortunately, most patients are able to lead a normal life with it for many years, and in some cases, it goes into remission on its own.

People with IgA nephropathy may need to take medications that suppress their immune system and thus have to be careful about infection. Despite this, the condition can typically be managed for a long time.


What autoimmune diseases cause kidney problems? ›

Lupus nephritis occurs when lupus autoantibodies affect structures in your kidneys that filter out waste. This causes kidney inflammation and may lead to blood in the urine, protein in the urine, high blood pressure, impaired kidney function or even kidney failure.

Is autoimmune kidney disease curable? ›

If you progress to end-stage kidney disease, you may be treated with dialysis or a kidney transplant. While kidney transplant is an option, it's not considered a cure, as the IgA nephropathy can reoccur in the newly transplanted kidney.

What are the 3 early warning signs of kidney disease? ›

Here are three signs that could indicate that you are beginning to experience a decline in kidney function.
  • Dizziness and Fatigue. One of the first possible signs of weakening kidneys is the experience of overall weakness in yourself and your overall health. ...
  • Swelling (Edema) ...
  • Changes in urination.

Is renal disease an autoimmune disease? ›

Inflammatory renal disease in the context of autoimmunity occurs because the kidney is targeted by effector responses. The effectors of autoimmunity in the kidney are many, but most often disease is initiated either by antibody deposition or infiltration of immune cells.

How do you treat autoimmune kidney disease? ›

Treatments include medicine that calms down the immune system and a blood-cleaning therapy (called plasmapheresis) to remove the autoantibodies. In this rare disease, irritation and swelling of the blood vessels (vasculitis) damages different organs in the body.

What does kidney inflammation feel like? ›

Pain in your sides, under your rib cage or in your abdomen. Severe or sharp pain that comes in waves. Pain that spreads to your groin area. Kidney pain is often accompanied by nausea or vomiting, especially if the pain is due to kidney stones.

Can kidney disease go back to normal? ›

While it's not possible to reverse kidney damage, you can take steps to slow it down. Taking prescribed medicine, being physically active, and eating well will help. You'll also feel better and improve your overall well-being.

What is the most life threatening autoimmune disease? ›

Four of the most frequently fatal ones include:
  • Giant cell myocarditis.
  • Anti-NMDA receptor encephalitis.
  • Mixed connective tissue disease.
  • Autoimmune vasculitis.
Oct 16, 2022

Can you get rid of autoimmune disease? ›

Autoimmune disorders in general cannot be cured, but the condition can be controlled in many cases. Historically, treatments include: anti-inflammatory drugs – to reduce inflammation and pain. corticosteroids – to reduce inflammation.

What foods help repair kidneys? ›

The Best Foods for Kidney Health
  • Dark leafy greens. Dark leafy green vegetables such as kale, spinach, chard, and collard greens are loaded with vitamins A and C, calcium, and many other important minerals. ...
  • Berries. ...
  • Cranberries. ...
  • Sweet potatoes. ...
  • Olive oil. ...
  • Fatty fish. ...
  • Cabbage.

Where do you itch with kidney disease? ›

Itching with kidney disease can be generalized, or it can occur in specific parts of the body. Common areas for this type of itching include the head, arms, back, and abdomen. It also tends to be worse at night, which can disturb your sleep.

What is the biggest indicator of kidney disease? ›

Glomerular filtration rate is the best overall indicator of kidney function. It is superior to the serum creatinine level, which varies with age, sex, and race and often does not reflect kidney function accurately.

What organ is affected by autoimmune disease? ›

Areas often affected by autoimmune disorders include: Blood vessels. Connective tissues. Endocrine glands such as the thyroid or pancreas.

What is renal disease life expectancy? ›

Many people with ESRD who receive dialysis regularly or have a kidney transplant can often live long, healthy, active lives. The life expectancy for a person receiving dialysis is around 5–10 years, though many live for 20–30 years.

What does it mean to live with an autoimmune disease? ›

Autoimmune Disease Basics

Autoimmune disease happens when the body's natural defense system can't tell the difference between your own cells and foreign cells, causing the body to mistakenly attack normal cells. There are more than 80 types of autoimmune diseases that affect a wide range of body parts.

How do you stop autoimmune disease? ›

Eating a well-balanced diet and getting regular exercise may also help you feel better. BOTTOM LINE: The main treatment for autoimmune diseases is with medications that bring down inflammation and calm the overactive immune response. Treatments can also help relieve symptoms.

Can I live a normal life with IgA nephropathy? ›

There's no cure for IgA nephropathy and no reliable way to tell how it will affect each person individually. For most people, the disease progresses very slowly. Up to 70 percent of people can expect to have a normal life expectancy without complications.

Can you make kidney disease go away? ›

There's no cure for chronic kidney disease (CKD), but treatment can help relieve the symptoms and stop it getting worse. Your treatment will depend on the stage of your CKD. The main treatments are: lifestyle changes – to help you stay as healthy as possible.

What test shows kidney inflammation? ›

A urinalysis can reveal signs of poor kidney function, such as red blood cells and proteins that should not be in urine or white blood cells that are a sign of inflammation.

What diseases cause inflammation of the kidneys? ›

Inflammation Diseases of the Kidneys
  • Glomerulonephritis. ...
  • Membranoproliferative glomerulonephritis (MPGN) ...
  • Interstitial nephritis. ...
  • IgA nephropathy (Berger's disease) ...
  • Pyelonephritis. ...
  • Autoimmune disorders related to CKD and inflammation. ...
  • Lupus nephritis. ...
  • Goodpasture's syndrome.

What disorder causes kidney inflammation? ›

Glomerulonephritis (gloe-MER-u-loe-nuh-FRY-tis) is inflammation of the tiny filters in the kidneys (glomeruli). The excess fluid and waste that glomeruli (gloe-MER-u-lie) remove from the bloodstream exit the body as urine. Glomerulonephritis can come on suddenly (acute) or gradually (chronic).

What improves kidney function? ›

Drink plenty of fluids

Regular, consistent water intake is healthy for your kidneys. Water helps clear sodium and toxins from your kidneys. It also lowers your risk of chronic kidney disease. Aim for at least 1.5 to 2 liters in a day.

What is stage1 kidney disease? ›

In Stage 1 CKD, the damage to your kidneys is mild. Your kidneys are still working well, but you may have signs of kidney damage or physical damage to your kidneys. Stage 1 CKD means you have a normal estimated glomerular filtration rate (eGFR) of 90 or greater, but there is protein in your urine (i.e., your pee).

Can kidney disease cause weight gain? ›

When kidneys do not work well, they do not make enough urine to remove the extra fluid from the body, causing fluid weight gain.

How do doctors check for autoimmune diseases? ›

Tests that may be done to diagnose an autoimmune disorder include: Antinuclear antibody (ANA) tests. Autoantibody tests. Complete blood count (CBC) with white blood cell differential (CBC with WBC differential)

Why do you get autoimmune disease? ›

No one is sure what causes autoimmune diseases. In most cases, a combination of factors is probably at work, such as: Genes, which may make you more likely to develop the disease. Environment, such as a virus that triggers the disease if you have the gene(s).

How do you detect autoimmune disease? ›

Blood Tests for Autoimmune Diseases
  1. C-Reactive Protein (CRP)
  2. Erythrocyte Sedimentation Rate (ESR)
  3. Antinuclear Antibodies (ANA)
  4. Ferritin.
  5. Enzyme-linked Immunosorbent Assay (ELISA)
  6. Rheumatoid Factor (RF)
  7. Anti-cyclic Citrullinated Peptide (Anti-CCP) Antibodies.
  8. Immunoglobulins.
Dec 5, 2022

Can vitamin D reverse autoimmune disease? ›

Vitamin D supplements lower risk of autoimmune disease, researchers say. “This is the first direct evidence we have that daily supplementation may reduce AD incidence, and what looks like more pronounced effect after two years of supplementation for vitamin D,” said Karen Costenbader, senior author of the study.

What foods trigger autoimmune disease? ›

Foods such as red meat, dairy, pastry, and beverages containing caffeine and alcohol trigger systemic inflammation, aggravating the autoimmune disease. If you are struggling with this condition, check out the most common foods that worsen autoimmune diseases so you can avoid them altogether.

What foods heal autoimmune? ›

Good foods

Antioxidant foods: Berries, green leafy vegetables, citrus fruits, cruciferous vegetables (kale, cabbage, etc.), beans, tomatoes and beets. Omega-3 fatty acids: Olive oil, fish oil, flax seeds, chia seeds, walnuts or soybeans.

What is harmful to kidneys? ›

The two most common conditions that affect your kidneys are diabetes and high blood pressure. A balanced diet and regular exercise can help keep both under control. With diabetes, it's also important to keep a close eye on your blood sugar and take insulin when you need it.

Is coffee good for the kidneys? ›

Studies also show that coffee has protective effects on the kidneys, thanks to antioxidants. Drinking coffee is associated with a lower risk of developing chronic kidney disease (CKD).

What color is your pee if you have kidney disease? ›

When kidneys are failing, the increased concentration and accumulation of substances in urine lead to a darker color which may be brown, red or purple. The color change is due to abnormal protein or sugar, high levels of red and white blood cells, and high numbers of tube-shaped particles called cellular casts.

What do fingernails look like with kidney disease? ›

Nail changes.

People who have advanced kidney disease can develop: A white color on the upper part of one or more nails and a normal to reddish brown color below, as shown here (half-and-half nails) Pale nails. White bands running across one or more nails (Muehrcke's nails)

What do renal failure nails look like? ›

White streaks, also called Muehrcke's nails,⁷ are common in people with kidney disease. Muehrcke's nails are white streaks that run parallel to the knuckles of the fingernail. They form when there isn't enough blood flow to the nail bed.

What is red flags in kidney disease? ›

Reduced GFR is a red flag for six major complications in patients with CKD: acute kidney injury risk, resistant hypertension, metabolic abnormalities, adverse drug reactions, accelerated cardiovascular disease and progression to end-stage kidney disease.

How long can you have kidney disease without knowing? ›

Around 90 percent of people who have this condition are unaware they have it. 2 out of 5 adults who have CKD don't know they have severe chronic kidney disease. People with CKD can live for years without knowing, as it doesn't always have the most clearly defined symptoms.

What test can confirm kidney disease? ›

Urine albumin-to-creatinine ratio (UACR).

A UACR test lets the doctor know how much albumin passes into your urine over a 24-hour period. A urine albumin test result of 30 or above may mean kidney disease. It's important to know that: The test may be repeated once or twice to confirm the results.

What 2 things are responsible for autoimmune disease? ›

Three factors are at play in the pathogenesis of autoimmune diseases: genes, immune system, and the environment where the patient lives. The genes confer what is called "predisposition" or genetic susceptibility. The immune system becomes dysregulated and provides the tools for executing the pathological damage.

What are the 7 autoimmune diseases? ›

Examples of autoimmune diseases include:
  • Rheumatoid arthritis. ...
  • Systemic lupus erythematosus (lupus). ...
  • Inflammatory bowel disease (IBD). ...
  • Multiple sclerosis (MS). ...
  • Type 1 diabetes mellitus. ...
  • Guillain-Barre syndrome. ...
  • Chronic inflammatory demyelinating polyneuropathy. ...
  • Psoriasis.

What happens if autoimmune is left untreated? ›

In fact, if an autoimmune disorder is left untreated, it can lead to more serious complications and even death. The person will also run a higher risk of infections.

What diseases commonly affect the kidneys? ›

Diabetes is the most common cause of kidney disease. Both type 1 and type 2 diabetes. But also heart disease and obesity can contribute to the damage that causes kidneys to fail. Urinary tract issues and inflammation in different parts of the kidney can also lead to long-term functional decline.

Which common medical condition is the most frequent cause of the kidney disease? ›

In the United States, diabetes and high blood pressure are the leading causes of kidney failure, accounting for 3 out of 4 new cases.


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2. The enzyme that could help 700 million people with chronic kidney disease
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3. Chronic Kidney Disease (CKD) | Etiology, Pathophysiology, Clinical Features, Diagnosis, Treatment
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4. Anemia from Chronic Kidney Disease (CKD)
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