Complications Complications of IgA Nephropathy (IgAN) IgA nephropathy damages the kidneys and causes blood and protein to leak into the urine. Over time, it can lead to complications like these. Chronic Kidney Disease (CKD) The risk is higher in people who have another condition that harms the kidneys, such as diabetes or high blood pressure.e60dc2a1-f33c-4a05-9b50-8e3e8e597629c10a59a7-fed6-4113-88b9-9ba0d6eac331 Nephrotic Syndrome Damage to the kidneys’ filters causes a group of problems, such as high protein in the urine, high cholesterol, and swelling.e60dc2a1-f33c-4a05-9b50-8e3e8e597629a7adca85-200d-495a-84b1-4275d4c21235 High Blood Pressure Damaged kidneys don’t control blood pressure as effectively as healthy kidneys.e60dc2a1-f33c-4a05-9b50-8e3e8e597629ffe459a2-7bdb-4e38-9485-86dddaa73531 Cardiovascular Disease People with IgAN have high blood pressure and high cholesterol, which increase their risk for a heart attack, stroke, and heart failure.e60dc2a1-f33c-4a05-9b50-8e3e8e597629fc4aabaa-36b7-41a3-801e-84acd05f00fb Kidney Failure These organs can become so damaged that they can no longer filter the blood.
Treatment Treatment and Medication Options for IgA Nephropathy (IgAN) There’s no cure for IgAN. Treatments reduce protein in the urine, slow kidney damage, relieve symptoms, and prevent complications.e60dc2a1-f33c-4a05-9b50-8e3e8e597629da8e4f52-71d9-4617-b423-fd180a446b54 A doctor’s recommendations will depend on the extent of your kidney damage and what other health conditions you have.e60dc2a1-f33c-4a05-9b50-8e3e8e5976292c255105-6b1a-4bb7-984d-62b89f89b63d Medication Options These are some of the medicines used to treat IgAN. Blood Pressure Medicines Angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs) are usually the first treatments doctors prescribe. These medicines lower blood pressure, reduce urinary protein levels, and slow the progression of IgAN.e60dc2a1-f33c-4a05-9b50-8e3e8e597629a82f31e0-c4a6-4163-be27-72b425cc29e0 Sodium-Glucose Cotransporter 2 (SGLT2) Inhibitors This class of medications was developed for diabetes, but they also treat IgAN. SGLT2 inhibitors reduce protein in the urine and slow the disease.e60dc2a1-f33c-4a05-9b50-8e3e8e5976298ec9ca9b-ad84-40ef-b5c3-bdacf074eb01 Endothelin Receptor [Type A] Antagonists (ERAs) This is a new type of medicine for IgAN. Atrasentan (Vanrafia) and sparsentan (Filspari) widen blood vessels in the kidneys. They reduce the amount of protein lost in the urine and preserve kidney function.e60dc2a1-f33c-4a05-9b50-8e3e8e5976290600b91e-9c5f-465e-be4f-5955295d6692 Corticosteroids (Steroids) These calm the immune system so it produces less IgA and reduce the amount of protein in the urine. Taking a steroid medicine for six to nine months can slow disease progression. Doctors don’t recommend staying on the medicine for longer because of serious side effects like high blood sugar, infections, and weak bones. The newer targeted-release steroid budesonide (Tarpeyo) has fewer side effects.e60dc2a1-f33c-4a05-9b50-8e3e8e5976296c4bdc07-1695-44ec-a424-276e3cb60159 Complement Factor B Inhibitor This type of drug targets a specific part of the immune system to reduce protein levels in the urine and slow disease progression. Iptacopan (Fabhalta) is the only drug approved in this class.e60dc2a1-f33c-4a05-9b50-8e3e8e5976291e954db6-cf8d-4432-9439-2ff3b56e6749 Monoclonal Antibody A four-weekly injected medication called sibeprenlimab-szsl (Voyxact) is one of the more recently approved treatments for IgAN.e60dc2a1-f33c-4a05-9b50-8e3e8e59762974171e9e-68ed-42ba-b73f-cb092428f89f Sibeprenlimab-szsl targets and blocks a protein called a proliferation-inducing ligand (APRIL) that normally drives the production of IgA antibodies in certain immune cells. This reduces the amount of IgA being produced, which, in turn, reduces kidney inflammation and the amount of protein in urine.e60dc2a1-f33c-4a05-9b50-8e3e8e5976294ec0091d-48ac-49b1-9c48-54c4d68742fc Surgery Surgery is not a standard treatment for IgA nephropathy, but tonsillectomy and kidney transplants may occur for certain people. Tonsillectomy The tonsils produce some of the body’s IgA, and IgA levels often decrease after tonsil removal. Some studies, most of which took place in Asia, found that tonsillectomy slowed the progression of IgA nephropathy. Because more rigorously designed studies haven’t confirmed these findings, doctors don’t recommend tonsillectomy for most people with IgAN.e60dc2a1-f33c-4a05-9b50-8e3e8e597629c9058a4d-0194-49fb-9c22-dd1c86ad3e37 But this surgery may be helpful alongside steroids for children with severe IgAN.e60dc2a1-f33c-4a05-9b50-8e3e8e597629262cd31f-a951-44dd-aae0-eb001e49193c Kidney Transplant IgA nephropathy is a progressive disease. Up to half of people with this condition develop kidney failure within 10 years after diagnosis.e60dc2a1-f33c-4a05-9b50-8e3e8e5976290b0e6207-58dd-4e8d-a308-d611054c0888 A kidney transplant is an option, but the disease can come back in a transplanted kidney. One study found that nearly 1 in 4 people had a recurrence of IgAN within 15 years after their kidney transplant.e60dc2a1-f33c-4a05-9b50-8e3e8e5976299e422ed4-af13-4540-8e32-c1e28f6fe2db
Symptoms Signs and Symptoms of IgA Nephropathy (IgAN) Symptoms of IgA nephropathy (IgAN) can take years to become noticeable.e60dc2a1-f33c-4a05-9b50-8e3e8e59762947e231ee-8950-4a97-9d10-2513fc6072ba The main symptom is blood in the urine, which looks brown, red, or cola-colored. This blood leaks into the urine from damaged blood vessels in the kidneys.e60dc2a1-f33c-4a05-9b50-8e3e8e59762980d9960f-a561-437f-b7ae-9cc166f62de3 Other common symptoms and signs include the following:e60dc2a1-f33c-4a05-9b50-8e3e8e597629998952a2-5515-4e87-8d7e-8012ce1ee919 Foamy urine (a sign of too much protein in the urine) Swelling in the face, belly, legs, and ankles Pain on the sides of the lower back Rash High blood pressuree60dc2a1-f33c-4a05-9b50-8e3e8e597629f066ad02-5413-4f64-8ebb-dd1ed51a3fbc Tirednesse60dc2a1-f33c-4a05-9b50-8e3e8e59762938e750c4-7fdd-4fca-bdb2-ee9317dad88c Weaknesse60dc2a1-f33c-4a05-9b50-8e3e8e59762914c2ac2e-3107-4ddb-b24b-0e84f666fbcc
Diagnosis How Is IgA Nephropathy (IgAN) Diagnosed? The diagnosis starts with a physical exam. A kidney specialist (nephrologist) will ask about symptoms like blood in the urine, swelling, and foamy urine. They’ll also take a blood pressure reading, since high blood pressure is a common sign of IgAN.e60dc2a1-f33c-4a05-9b50-8e3e8e59762931b69c80-a75c-4e03-b0bd-fc740541dcfd These lab tests check for signs of kidney damage and kidney function.e60dc2a1-f33c-4a05-9b50-8e3e8e5976291c880071-ab81-42a3-91da-c1568d50b2b3 Urinalysis helps doctors identify blood and protein in the urine. Urine tests detect protein and the waste product creatinine in the urine. Blood tests check creatinine levels and measure the estimated glomerular filtration rate (eGFR), which shows how well your kidneys are filtering blood. A kidney biopsy confirms the diagnosis. For this test, the doctor removes a small piece of the kidney. A lab examines the sample using a special magnifying glass or a technique called immunofluorescence to determine whether the kidney tissue contains IgA deposits.e60dc2a1-f33c-4a05-9b50-8e3e8e5976297a31591d-4409-4218-ba1a-cb34774c6403
Support Support for People With IgA Nephropathy (IgAN) IgA Nephropathy Foundation This is the only organization that’s solely dedicated to helping people with IgAN and their caregivers. The IgA Nephropathy Foundation funds research to find a cure for this condition. It also offers information and a variety of support services.e60dc2a1-f33c-4a05-9b50-8e3e8e5976298ced69a5-c63d-4cc0-887c-749eafbd86e5 National Kidney Foundation The National Kidney Foundation is working to stop preventable types of kidney disease. People with IgAN and their caregivers can find information about treatments, resources, and clinical trials on the organization’s website.e60dc2a1-f33c-4a05-9b50-8e3e8e597629f57e1c6e-e2f7-4080-b551-241f75aed957
Types Types of IgA Nephropathy (IgAN) Doctors divide IgAN into three types, based on the cause and where in the kidneys the antibodies collect. Primary IgA Nephropathy This is the most common type. It starts in the kidneys, and no other condition causes it. In primary IgAN, the body produces too much galactose-deficient IgA1 (Gd-IgA1), a type of IgA. In response, the immune system produces antibodies that attach to Gd-IgA1. When the attached antibodies and Gd-IgA1 deposit in the kidneys, the immune system releases chemicals that inflame and irritate the kidneys’ filters.e60dc2a1-f33c-4a05-9b50-8e3e8e597629118f008c-8f41-4832-a116-d9f65d8d6002 Secondary IgA Nephropathy This type starts when a condition, such as liver disease or another autoimmune disease, prevents the body from clearing IgA. People with this type have symptoms of both IgAN and the underlying condition.e60dc2a1-f33c-4a05-9b50-8e3e8e59762951a2ffb1-e419-4b2c-9946-634f71a697d6 IGA-Associated Nephropathy In this group of diseases, IgAN affects the kidneys and other organs. One common form is IgA vasculitis , formerly known as Henoch-Schönlein purpura. IgA builds up in small blood vessels in the kidneys and other parts of the body. People with IgA vasculitis sometimes have damage to their gastrointestinal tract, joints, and kidneys.e60dc2a1-f33c-4a05-9b50-8e3e8e59762967e443db-d7af-40cb-ae43-0114d0fd9b74
IgA nephropathy (IgAN, or Berger’s disease) is an autoimmune disease that causes immunoglobulin A (IgA) antibodies to build up in the kidneys. Antibodies are proteins that the immune system makes to protect the body against bacteria and viruses. But a buildup of IgA antibodies damages the glomeruli, or vessels in the kidneys that filter blood, causing them to leak blood and protein into the urine.e60dc2a1-f33c-4a05-9b50-8e3e8e597629f8b5fe31-2766-4488-be1b-f7aa75914756 This condition progresses differently in each person who has it. Some people with IgAN live for many years without any problems. Others develop complications like high blood pressure , chronic kidney disease , or kidney failure.e60dc2a1-f33c-4a05-9b50-8e3e8e5976298b348d90-eb81-4f4a-8436-8d73811d5e86 IgAN has no cure, but treatment aims to slow the progression of kidney disease and improve quality of life by managing symptoms.e60dc2a1-f33c-4a05-9b50-8e3e8e597629c144ac4d-2fc8-4f63-a200-15be0aa82bed
Prognosis IgA Nephropathy (IgAN) Prognosis IgAN is a lifelong, progressive disease. Eventually, it can lead to kidney failure, which is life-threatening.e60dc2a1-f33c-4a05-9b50-8e3e8e597629a9f76e93-3a57-4875-ace2-aa3f5f74d47b Up to half of people with this condition develop kidney failure within 10 years after they’re diagnosed.e60dc2a1-f33c-4a05-9b50-8e3e8e59762991b1076a-7510-43da-8b07-1519c988a2f0 Every person with IgAN experiences a different disease progression. Some people respond well to treatment and notice that their symptoms improve. Others progress to kidney failure in a few years.e60dc2a1-f33c-4a05-9b50-8e3e8e59762938c595a1-943d-424f-846d-c15ed11eba64 High levels of protein in your urine increases the risk of kidney failure within 10 years, according to research.e60dc2a1-f33c-4a05-9b50-8e3e8e597629321072f9-0548-451d-a777-c705d22809ae Once you have kidney failure, you’ll need dialysis or a kidney transplant.e60dc2a1-f33c-4a05-9b50-8e3e8e597629523c3039-09bc-4f21-9fa7-a35986bf6808 IgA nephropathy isn’t curable, but treatments can extend your lifespan and improve your quality of life. The right treatment plan will help protect your kidneys and slow the disease.e60dc2a1-f33c-4a05-9b50-8e3e8e5976290e9a150f-d915-4124-8e15-8c9d560063a8
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Lifestyle Lifestyle Changes for IgA Nephropathy (IgAN) Staying generally healthy will also protect your kidneys, lower your blood pressure, and support the management of other medical conditions.e60dc2a1-f33c-4a05-9b50-8e3e8e5976291dbf91ce-ee5f-4aed-9dea-aa9719544fe4 Your doctor may recommend the following lifestyle changes to manage IgAN and prevent it from getting worse. Make Changes to Your Diet Try to follow a balanced diet. Your doctor or a registered dietitian can help you choose a nutritious mix of foods. A few dietary changes are important for IgAN. Limit sodium to 2,000 milligrams (about 1 teaspoon) or less per day. Salt raises blood pressure and increases the amount of protein released in urine.e60dc2a1-f33c-4a05-9b50-8e3e8e59762917e03f2c-e098-4135-9987-e866c817352b Reduce protein in your diet. You need some protein to keep your body functioning optimally, but too much can be hard on damaged kidneys.e60dc2a1-f33c-4a05-9b50-8e3e8e5976295f69ee3d-add8-4c24-a9f4-a16ad5570327 Avoid alcohol or limit drinking. Alcohol can raise your blood pressure. It also interacts with some blood pressure medicines in ways that can cause your pressure to rise too high or drop too low.e60dc2a1-f33c-4a05-9b50-8e3e8e59762934307e9a-9f3e-40eb-bdde-16731c0b2749 Avoid Tobacco Smoke Smoking is generally harmful to your body, with links to cancer , heart disease, and other conditions. Tobacco smoke can speed up IgAN kidney damage. This, in turn, increases your risk for kidney failure. If you’ve had trouble quitting , ask your doctor about medications and other tools to help you stop smoking.e60dc2a1-f33c-4a05-9b50-8e3e8e59762980ca201f-d476-4c1c-b73c-697375d25a70 Take Care With Medicines That Harm the Kidneys Some medicines protect the kidneys, while others can cause further damage. These are a few drugs to limit or avoid:e60dc2a1-f33c-4a05-9b50-8e3e8e597629ebc1466b-e724-4295-9a18-163439640aed Blood Thinners apixaban (Eliquis), dabigatran (Pradaxa), rivaroxaban (Xarelto), and warfarin (Coumadin) Diabetes Drugs glyburide (Diabeta, Glynase), glimepiride (Amarl), insulin, and metformin (Glucophage) Heartburn Medicines proton pump inhibitors (PPIs) like esomeprazole (Nexium), lansoprazole (Prevacid), and omeprazole (Prilosec) and H2 blockers like cimetidine (Tagamet) and famotidine (Pepcid) Nonsteroidal Anti-Inflammatory Drugs (NSAIDs) ibuprofen (Advil, Motrin), naproxen sodium (Aleve), and aspirin Other Medications certain antibiotics, antifungal drugs, and antiviral drugs Many medications remain safe for people with long-term kidney problems at a lower dose or with less frequent use. Speak to your doctor about the right medication and dosage for your eGFR and medical needs.e60dc2a1-f33c-4a05-9b50-8e3e8e5976295b70a903-7900-4948-9d8d-7c2bc0bdd220
Causes Causes and Risk Factors of IgA Nephropathy (IgAN) Primary IgA nephropathy (IgAN) occurs when an abnormal immune response causes immunoglobulin A (IgA) to deposit in the glomeruli. The condition often starts after a cold or other respiratory infection. As the immune system fights off the virus or bacteria that caused the infection, it makes too much IgA. The extra IgA travels through the blood and gets stuck in the kidneys’ filters, causing inflammation and damage.e60dc2a1-f33c-4a05-9b50-8e3e8e59762941895fc8-ff3c-4220-b01b-f70cf176795e IgAN isn’t an infection. Instead, it results from a problem with the immune system. In fewer than 10 percent of people with primary IgAN, genetic changes cause the immune problem, but in the remaining 90 percent, no obvious cause is apparent.e60dc2a1-f33c-4a05-9b50-8e3e8e5976294d317bf0-eb9b-41c2-8369-e47c46b3c06a Secondary IgAN occurs when another condition causes excessive IgA to build up in the blood. Diseases linked to this form of IgAN include:e60dc2a1-f33c-4a05-9b50-8e3e8e597629c63f4ac6-7a10-4477-8919-cce701472365 Liver diseases such as cirrhosis Autoimmune diseases such as Sjögren’s syndrome , spondyloarthritis , lupus , and an inflammatory blood vessel condition called Behçet’s disease Celiac disease Inflammatory bowel disease (IBD) Psoriasis Viral infections like COVID-19 , HIV , and hepatitis B The following factors may increase your risk for IgAN: A family history of IgAN or IgA vasculitise60dc2a1-f33c-4a05-9b50-8e3e8e5976297d50b147-55d9-4bfd-91ee-db7cdad6a597 Male sex; IgA nephropathy affects twice as many males as females in Europe and North Americae60dc2a1-f33c-4a05-9b50-8e3e8e59762970749bf2-3f38-446b-b3ee-f325c4a76243 A history of celiac disease, IBD, hepatitis, cirrhosis, or HIVe60dc2a1-f33c-4a05-9b50-8e3e8e5976291529966f-1222-4428-b107-516c7c43cb83 Age between 10 and 40e60dc2a1-f33c-4a05-9b50-8e3e8e597629afcd6f95-0202-4813-b0d7-6ae5002b50f0 Asian, Pacific Islander, or white race or ethnicity e60dc2a1-f33c-4a05-9b50-8e3e8e597629f693f6a2-8c06-4e97-b644-7a3f94948326
Clinical Trials Researchers are studying new treatments for IgAN in clinical trials. They’re investigating different combinations of medicines and learning how certain drugs affect specific groups of people. Joining a study of an investigational drug could give you a chance to try a new therapy before it’s available to the public.e60dc2a1-f33c-4a05-9b50-8e3e8e597629d79618de-16e7-4090-85e3-b169fbf4ac61
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