Pulmonary vasculitis

This page has information for parents on pulmonary vasculitis in children.


What is pulmonary vasculitis?

Vasculitis is inflammation of the blood vessels.

Pulmonary – to do with the lungs

Vasculitis – inflamed blood vessels

There are many different types of vasculitis, affecting different parts of the body. Pulmonary vasculitis is the name we use to describe vasculitis when it affects the lungs.

Blood vessels are the tubes that carry blood around the body.

These tubes vary in size. The main vessel carrying blood from the heart is about 30cm long and 2.5cm wide. Other tiny blood vessels can only be seen under a microscope. Vasculitis can happen in any blood vessel.


What causes pulmonary vasculitis?

In pulmonary vasculitis, the body’s immune system doesn’t work as it should. The body’s usual defence systems are designed to fight off infections and keep us well. Instead they start fighting other parts of the body. We don’t know exactly why this happens.

When blood vessels are inflamed, they may be damaged, and their walls may become stretched and thin. This can make the blood vessels fragile. Some people may have genes passed down from their parents that make it more likely.

A variety of very rare conditions may cause pulmonary vasculitis. Some examples of pulmonary vasculitis affecting children include:

  • Granulomatosis with polyangiitis (GPA) – this used to be called Wegener’s granulomatosis. It can affect the lungs and airways, but also often affects the nose, eyes, ears and kidneys.
  • Eosinophilic granulomatosis with polyangiitis (EGPA) – this used to be called Churg-Strauss syndrome. It most commonly affects the lungs and skin, but can also affect blood vessels in other parts of the body. Children with EGPA may have been treated for asthma before EGPA was suspected.

Who’s at risk of pulmonary vasculitis?

Pulmonary vasculitis is very rare. We don’t know exactly how many children are affected. Around 2 children a year begin treatment for GPA at Great Ormond Street Hospital.  


What are the symptoms of pulmonary vasculitis?

Because pulmonary vasculitis can affect different parts of the body, symptoms will vary. Symptoms affecting the lungs may include:

If you’re worried about any of your child’s symptoms, seek medical help. Find out about seeing a doctor and when to call 999 about your child’s breathing.

Your child may also have more general symptoms:

  • weight loss
  • poor appetite
  • fever
  • tiredness.
  • a rash: if the skin is involved, they may have a rash.
  • blood in their urine: if the kidneys are affected, your child may have blood in their urine.

Your child may have been diagnosed with another lung condition, such as asthma. If they aren’t responding to treatment, it’s important to consider pulmonary vasculitis, even though it’s very rare.


How is pulmonary vasculitis diagnosed?

Your child will have blood tests and a urine test.  They may need one or more tests to look at the lungs in more detail:

  • an X-ray or CT scan. Doctors will look for inflamed blood vessels or areas of bleeding
  • bronchoscopy. A narrow, flexible tube with a camera on the end is used to look inside your child’s lungs
  • lung biopsy. A surgeon removes some tissue from the lung under general anaesthetic. This can sometimes be done using keyhole surgery. If doctors think your child’s kidneys might be affected, they may also have a kidney (renal) biopsy.

How is pulmonary vasculitis treated?

Pulmonary vasculitis is a long-term condition with no cure. It affects each person differently.

Although there’s no cure, it can be treated by reducing inflammation and suppressing the immune system. It’s important that this happens as soon as possible. Prompt treatment can prevent or limit long term damage to the lungs, and any other organs involved.

Children diagnosed with pulmonary vasculitis should be looked after by specialists. They can guide the correct treatment of this rare condition. They can also make sure families are aware of clinical trials and any new treatments.

Your child will be given steroid medicine to reduce inflammation.

They will also have medicine to suppress the immune system. There are different types of medicine that may be used. Your child’s treatment will depend on how severe their condition is, and what has caused it.

Your child’s specialist doctors will talk to you in detail about these medicines before starting treatment.


Where can I find out more?

Vasculitis UK has more information on living with vasculitis.

If you have concerns or need advice, call our helpline on 03000 030 555 between 9am and 5pm on a weekday or email them.

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Last medically reviewed: September 2019. Due for review: September 2022

This information uses the best available medical evidence and was produced with the support of people living with lung conditions. Find out how we produce our information. If you’d like to see our references get in touch.