Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura or HSP, is a rare condition that can cause chronic kidney disease (CKD). It occurs when immunoglobulin A, an immune system product or antibody, collects in small blood vessels, which then become inflamed and leak blood. Learn more about IgA Vasculitis including the symptoms, causes, diagnostic tools, and treatment.* 

What Is IgA Vasculitis? 

IgA vasculitis is a form of blood vessel swelling that mainly affects small vessels, or capillaries, in the skin and kidneys, and is caused by an abnormal immune system response in the body. 

Who is most likely to develop IgA vasculitis?

With up to 27 cases per 100,000 children and infants, and fewer than 2 cases per 100,000 adults, IgA vasculitis is highly uncommon. It tends to be mostly seen in children ages 4 to 7. Individuals with a family history of the condition are also more likely to develop it. 

Is IgA Vasculitis a Long-Term Illness?

In most cases, IgA vasculitis goes away without treatment within a few weeks to a few months. In rare instances, IgA vasculitis can lead to CKD, a serious long-term condition. Adults are more likely than children to develop CKD, and warrant more aggressive treatment than children do. 

What Are the Symptoms of IgA Vasculitis?

The symptoms of IgA vasculitis are widespread, and can affect the skin, joints, bowels, and kidneys. Less often, it may also affect the nerves, brain, and lungs. 

The most common symptoms are:

• Skin rashes (though with no itching or pain)
• Abdominal pain
• Nausea and/or vomiting
• Joint pain and swelling (particularly in knees, ankles, hands, and wrists)

Less common signs and symptoms include:

• Hematuria (blood in the urine)
• Bowel obstruction
• Leg swelling
• Coughing
• Seizures
• Pneumonia 

What Causes IgA Vasculitis?

IgA vasculitis occurs from an autoimmune response, where the body’s immune system attacks its own cells and organs. While researchers are not completely sure what causes this response, they think it may be linked to:

• Bacteria and viruses
• Food allergies
• Immunizations
• Bites or stings from insects
• Medication allergies or side effects

Genetic factors can increase your risk of developing the conditions. 

What Tests Are Used to Diagnose IgA Vasculitis?

There is no single test that will diagnose IgA vasculitis. A doctor will use a combination of a person’s medical history, physical exam, and laboratory tests to diagnose. Some of the tests a suspected IgA vasculitis patient should expect include:

• Blood and urine tests
• Skin biopsy
• Kidney biopsy
• Ultrasound imaging

How Is IgA Vasculitis Treated? 

IgA vasculitis typically goes away on its own, and there is no specific treatment for it. Certain medications may be prescribed to alleviate symptoms such as abdominal pain, joint pain, and swelling. 

In instances where IgA vasculitis affects the kidneys, more aggressive treatment may be necessary to prevent CKD from occurring or getting worse. Corticosteroids and other immunosuppressive medications may be used to help reduce inflammation and further damage to the kidneys.  

Are clinical trials available for IgA vasculitis patients?

Since there are still many unknowns about IgA vasculitis, researchers need patient volunteers to help learn more about the condition. 

Researchers are studying several areas right now, including:

• causes of IgA vasculitis
• new treatments for the condition and its symptoms
• genes that increase the risk of developing IgA vasculitis
• characteristics and outcomes among pregnant women with this condition

If you or someone you know is interested in being a participant, speak with your healthcare provider and visit the National Institute of Health’s Recruitment website.  

*IgA Vasculitis. (2021, December 9). National Institute of Diabetes and Digestive and Kidney Diseases. https://www.niddk.nih.gov/health-information/kidney-disease/iga-vasculitis#

IgA Vasculitis (Formerly Henoch-Schönlein Purpura or HSP). (2018, April 6). UNC Kidney Center. https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/iga-vasculitis-formerly-henoch-schonlein-purpura-or-hsp/