This information is provided for educational and informational purposes only and does not constitute providing medical advice or professional services.
What are the kidneys and what do they do?
The kidneys are two bean-shaped structures located on either side of the backbone. Kidneys play a very important role in the human body.
Each kidney has a million little filters (called glomeruli) that act as a sieve. As blood flows through these filters, the good things (like red blood cells, protein, sugar, etc.) are retained while excess water and waste products are removed via urine.
The kidneys also maintain our blood pressure, provide building blocks to maintain our red blood cells (hemoglobin), and also activate vitamin D to maintain healthy bones.
What is IgA Nephropathy?
IgA nephropathy is a kidney disease that occurs when abnormal IgA proteins are produced by the body and are deposited in the filters (glomeruli) of our kidneys. IgA proteins (or IgA immunoglobulins) are proteins that are made by our immune system to fight infections. IgA deposition causes inflammation and can cause leakage of blood and protein in the urine. It eventually damages and scars the kidneys over time.
We do not fully understand what causes the production of the abnormal IgA proteins or why they only deposit in the kidneys’ filters. We know that there is an association with certain infections, liver disease, coeliac disease, and other conditions. There may also be a genetic predisposition to this disease.
Who is at risk for IgA Nephropathy?
IgA nephropathy is known to be more common among people of Asian (Far East) and European ancestry. It can occur at any age, but it is more commonly seen in younger ages starting in the teens to late 30s. IgA nephropathy occurs more often among males than females and those with a family history of this disease.
What are the symptoms of IgA Nephropathy?
Depending on your kidney function and the activity of the disease, you might experience different symptoms.
You may notice episodes of blood in the urine, where the urine appears tea-colored or like ‘coca-cola.’ This may often occur after a throat or stomach infection and sometimes after heavy exercise.
Often, blood in the urine may not be visible to your naked eye, but your doctor can perform a urine test that can detect small amounts of blood and protein in the urine. Sometimes this may only be discovered when you have a routine medical exam for other reasons.
You may also have high blood pressure, which can reflect kidney damage.
Sometimes, the disease presents as ‘IgA vasculitis’ and can involve other organs as well. In this case, you may notice a rash, especially on the legs, diarrhea, leg swelling, joint pains, and the features described above.
Some symptoms that may suggest the disease is at a late stage include nausea, vomiting, fatigue, loss of appetite, or leg swelling.
What tests will my doctor perform to diagnose IgA nephropathy?
Your doctor will probably request a blood test, urine test, ultrasound of the kidney, and often a kidney biopsy. A kidney biopsy provides a definitive diagnosis. No blood test can diagnose this condition.
How is IgA nephropathy treated?
Treatments will vary for each patient depending on the level of protein in your urine as well as the kidney biopsy report. There is no specific cure for IgA nephropathy, but we can take measures to slow the disease’s progression and damage to the kidneys.
Maintaining near-normal blood pressure and reducing the amount of protein leak in the urine are the cornerstones of current treatment.
Standard treatment options for IgA nephropathy include medications like angiotensin inhibitors or angiotensin II receptor blockers.
Some select patients may require treatments with steroids that are available under various formulations.
There is increasing evidence of using sodium-glucose cotransporter 2 inhibitors (SGLT2 inhibitors).
Your doctor may also prescribe cholesterol-lowering drugs such as statins if this is indicated.
If your kidney disease is very severe, your doctor will discuss your options for kidney replacement therapy (such as dialysis or a kidney transplant).
Leading a healthy lifestyle is important in supportive care treatment for IgA nephropathy. Your doctor will advise you to eat a balanced diet, reduce salt intake, exercise regularly, quit smoking, and reduce consumption of alcohol within recommended limits. It can be helpful to monitor your blood pressure at home regularly.
What is the long-term outcome of IgA nephropathy? Can I get kidney failure from IgA nephropathy?
Patients with little protein leak in the urine are at low risk for progressive kidney damage. However, some patients can develop kidney damage and increase protein leakage over time. Amongst these patients, 15-20% may develop advanced kidney failure by ten years and 20-30% by 20 years. IgA nephropathy can rarely be aggressive and cause a rapid loss of kidney function. If you develop kidney failure, you will need either dialysis or a kidney transplant.
It is difficult to predict what will happen in the future. Blood and urine tests are the primary way to detect how your disease is progressing, so regular checkups with your doctor are very important.
Are there any ongoing clinical trials for the treatment of IgA nephropathy?
Yes, several clinical trials are going on evaluating the efficacy of new agents for the treatment of IgA nephropathy. If you would like more information regarding the trials, you can find it here: www.enrollmypatient.org
Is there a way to prevent IgA nephropathy?
Currently, there is no proven method to prevent IgA nephropathy, but there are medications available that can delay the progression of the disease. If you have a family history of IgA nephropathy, talk to your doctor about how to keep your kidneys healthy.
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