Vasculitis in Children

What is vasculitis in children?

Vasculitis is inflammation (irritation and swelling) of your child’s blood vessels. Blood vessels are tubes within your body that carry blood through your organs and tissues. Vasculitis can affect very small blood vessels (capillaries), medium-sized blood vessels or large blood vessels like the main blood vessel that leaves your heart (aorta). There are several types of vasculitis, but IgA vasculitis is the most common among children.

What is IgA vasculitis?

IgA vasculitis (IgAV), or Henoch-Schönlein purpura, is inflammation that mainly affects the small blood vessels in your child’s skin, gastrointestinal tract (a series of hollow organs connected to each other from your mouth to your anus) and kidneys. IgA stands for immunoglobulin A. This is an antibody in your immune system that collects in blood vessels. Diagnostic tests can check for this immunoglobulin.

How common is vasculitis in children?

IgA vasculitis affects approximately 3 to 27 children out of every 100,000 in the United States and 20 children out of every 100,000 in the United Kingdom.

What are the symptoms of IgA vasculitis in children?

Symptoms of IgA vasculitis can affect many different parts of your child’s body, like their skin, joints, intestines and kidneys. Symptoms could include:

• A raised, discolored rash.
• Joint pain and swelling.
• Abdominal pain.
• Loss of appetite.
• Vomiting.
• Diarrhea and blood in pee or poop.
• Swelling of their scrotum and/or scalp.
• Fever.

What causes IgA vasculitis in children?

IgA vasculitis occurs when your child’s immune system attacks their blood vessels by mistake. Research suggests that IgAV may be an unusual reaction of your body’s immune system in response to:

• An infection (strep throat or viral illness).
• An insect bite.
• Certain foods.
• A vaccine.
• Certain medications.
• A genetic predisposition.

What happens to a child’s blood vessels with IgA vasculitis?

Vasculitis causes your child’s blood vessels to inflame. Inflammation occurs when your immune system sends out cells to attack bacteria or heal damaged tissue. Instead of targeting things that are unhealthy in your child’s body, their immune system attacks their healthy blood vessels. When inflammation happens, you’ll see and feel it as pain, swelling or skin discoloration.

During inflammation, the structure of your child’s blood vessels weakens. This is similar to a castle wall receiving damage after a battle. Weak blood vessels are more at risk of developing blood clots or narrowing. The damage to blood vessels can also create blockages where blood can’t easily move through. Less commonly, the vessels may become thin and stretch, which can lead to aneurysms or rupture.

The weak vessel wall itself becomes “leaky,” allowing the fluid from within the blood vessels to enter the surrounding tissues. This can cause swelling and other symptoms, like a skin rash.

Vasculitis can involve the blood vessels that supply blood to vital organs like your brain, kidneys, lungs or heart. Sometimes, this condition can compromise organ function and be very serious. Complications are more common among adults than children.

Is IgA vasculitis contagious?

No, IgA vasculitis isn’t contagious and doesn’t spread by person-to-person contact.

Who is at risk of developing IgA vasculitis?

While IgA vasculitis can affect anyone at any age, over 90% of people diagnosed with IgA vasculitis are children between the ages of 3 and 10 years old. IgAV affects more boys and children assigned male at birth (AMAB), but it can also affect girls and children assigned female at birth (AFAB). You’re more at risk of developing IgAV if someone in your biological family had the condition.

How is IgA vasculitis diagnosed?

A healthcare provider will diagnose IgA vasculitis after a physical exam and testing. During the exam, your child’s provider will ask questions about their symptoms and take a complete medical history. Tests to diagnose IgAV may include:

• Urinalysis.
• A complete blood count (CBC).
• Imaging tests of areas of their body with pain or symptoms.
• Lab tests to rule out other conditions with similar symptoms.

While less common, your healthcare provider may recommend a skin biopsy or kidney biopsy to look for a buildup of immunoglobulin A and further identify the severity of involvement at that site.

How is IgA vasculitis treated?

Treatment isn’t always necessary for IgA vasculitis because symptoms often go away on their own following the acute period. If your child’s symptoms are bothersome, a healthcare provider may recommend taking:

• Nonsteroidal anti-inflammatory drugs (NSAIDs) to treat joint pain, like naproxen.
• Corticosteroids like prednisone to treat abdominal pain, kidney involvement or significant swelling.

While IgAV is generally a rather mild illness that goes away on its own, it can also cause serious complications in the kidneys and bowels. Some children may need to take immunosuppressant medications to prevent these complications. Ask your child’s healthcare provider about the side effects of the medications they prescribe to understand how they may affect your child.

How soon after treatment will my child feel better?

Symptoms of IgA vasculitis can last two to 12 weeks, and often, symptoms go away after a month. While less common, symptoms may come back in the future.

Can vasculitis be prevented?

No, there’s no known way to prevent IgA vasculitis.

What can I expect if my child has vasculitis?

The majority of cases of IgA vasculitis go away within one month. Symptoms may be disruptive for several weeks, causing pain and discomfort, but treatment is available to relieve those symptoms and help your child feel better. Your child’s prognosis varies based on the extent of their symptoms and where in their body blood vessels inflame. While rare, long-term kidney damage can happen.

Children with IgA vasculitis shouldn’t have a major interference in their routine. Once your child’s symptoms resolve, they can go back to school, play sports and participate in extracurricular and family activities like normal. There aren’t specific limitations to physical activities for children with this condition as long as the activity is safe.

Is there a cure for IgA vasculitis?

No, there isn’t a cure available for IgA vasculitis. Research is ongoing to learn more about the condition.

When should my child see a healthcare provider?

Visit your child’s healthcare provider if they experience:

• Abnormal swelling
• Severe pain and discomfort.
• Symptoms that last longer than 12 weeks.
• Symptoms that go away and return.
• Symptoms that get worse instead of better.

What questions should I ask my child’s doctor?

• What over-the-counter pain medications are safe to give my child?
• How do I help my child eat if they don’t have an appetite?
• Will my child have long-term damage to their organs after their symptoms go away?
• Will my child’s symptoms come back in the future?

What is Kawasaki disease?

Kawasaki disease is a rare form of vasculitis that affects children. It causes a fever and inflammation of the blood vessels (mainly) of the heart; specifically, the coronary arteries that provide the heart with blood. Inflamed blood vessels may become weak and stretch in size. This can lead to coronary artery aneurysms.

A note from Cleveland Clinic

IgA vasculitis is a condition that can cause irritation and discomfort for a period of time (weeks) before your child feels better again. Most children make a full recovery without any long-term complications. While treatment isn’t always necessary, if your child is uncomfortable, visit their healthcare provider.