IgA Vasculitis?

What is immunoglobulin A (IgA) vasculitis?
IgA vasculitis, formerly called Henoch-Schönlein purpura or HSP, is a disease that causes the antibody immunoglobulin A to collect in small blood vessels, which then become inflamed and leak blood. Nearly all people with IgA vasculitis develop a red or purple rash NIH external link. Some people with IgA vasculitis also develop problems with their gastrointestinal (GI) tract, joints, and kidneys because of inflamed blood vessels in the intestines, joints, and kidneys. In rare cases, the lungs, nervous system, or other organs may be affected.

How common is IgA vasculitis?
IgA vasculitis is rare. The number of new cases of IgA vasculitis is approximately 3 to 27 cases per 100,000 in children and infants and fewer than 2 new cases per 100,000 each year in adults.1, 2

Who is more likely to develop IgA vasculitis?
IgA vasculitis is most common in young children between the ages of 4 and 7,3 but people of all ages can be affected.

You may be more likely to develop IgA vasculitis if you have a family history of the disease.

Are there long-term health problems from IgA vasculitis?
Most people recover from IgA vasculitis completely without treatment. The symptoms usually go away within a few weeks to months. The symptoms may return one or more times—usually within the first year—but typically go away again on their own.

The most common and serious long-term health problem caused by IgA vasculitis is CKD. Severe kidney damage is rare but, if it does occur, it may require aggressive treatment. Adults are much more likely than children to develop CKD.

How do health care professionals diagnose IgA vasculitis?
Health care professionals use your medical history, a physical exam, and lab tests to confirm your diagnosis of IgA vasculitis.

What tests do health care professionals use to diagnose or monitor IgA vasculitis?
Your health care professional may perform a skin or kidney biopsy, blood and urine tests, and sometimes an ultrasound of the kidneys or abdomen to confirm the diagnosis and monitor your health. In some cases, a kidney biopsy may be used to asses and monitor the severity of the disease. Depending on the organs affected, your health care professional may suggest other tests.

Skin biopsy
Your health care professional may obtain a skin biopsy to test for antibody deposits on your skin and confirm a diagnosis of IgA vasculitis. To do a skin biopsy, the health care professional removes skin cells from your body to examine under a microscope. You will be given a local anesthetic NIH external link so you don’t feel any pain.

Kidney biopsy
You may also need a kidney biopsy. A kidney biopsy can confirm the diagnosis of IgA vasculitis and provide information on the amount of kidney damage. Your health care professional will use the results of the kidney biopsy to help develop a treatment plan. The kidney biopsy is performed in a hospital with local anesthetic and, in some children, sedation.

Blood and urine tests
Your health care professional may use blood and urine tests to check the health of your kidneys. A urine sample can be used to determine whether there is blood or protein in your urine, which is a sign that the IgA vasculitis is affecting your kidneys. A blood test can check how well your kidneys are working.

Ultrasound
Depending on your symptoms, your health care professional may want you to have an ultrasound to check for GI or kidney problems.

Your health care professional will monitor your symptoms to determine if they are getting better. You may continue to have blood and urine tests to check your kidney function for at least 6 months after most of your symptoms disappear. Your health care professional will monitor you more closely if your tests show kidney damage, as this puts you at greater risk for developing CKD.

How do health care professionals treat IgA vasculitis?
There is no specific treatment for IgA vasculitis. The disease usually goes away on its own. However, your health care professional may suggest certain medicines to relieve symptoms such as abdominal pain, joint pain, and swelling. If your kidneys are involved, the treatment goal will be to prevent CKD.

If you are taking a medicine that may have caused the IgA vasculitis, you will stop taking that medicine.

How can I prevent IgA vasculitis?
Experts have not yet found a way to prevent IgA vasculitis. If your kidneys have been affected by the disease, treatments may be available to help prevent serious kidney problems. Talk with your health care professional about treatments, and follow the treatment plan your health care professional recommends.

How can my diet help prevent or relieve IgA vasculitis?
Diet and nutrition have not been shown to play a role in causing or preventing IgA vasculitis.

Clinical Trials for IgA Vasculitis
The NIDDK conducts and supports clinical trials in many diseases and conditions, including kidney disease. The trials look to find new ways to prevent, detect, or treat disease and improve quality of life.