Pulmonary vasculitis

Pulmonary = to do with the lungs

Vasculitis = inflamed blood vessels

Vasculitis is the name for a group of conditions that cause inflammation (swelling) of your blood vessels. Blood vessels vary in size and carry blood from your heart around your body. In vasculitis, your immune system attacks your blood vessels, making them swollen and narrow.

Vasculitis can happen in any blood vessel. There are many different types of vasculitis that affect different parts of the body.

Pulmonary vasculitis is the name given to vasculitis when it affects the lungs.

How common is pulmonary vasculitis?   

Pulmonary vasculitis is very rare. Around 10 to 15 people in a million are diagnosed with vasculitis every year.

If you or your child has pulmonary vasculitis, your immune system doesn’t work as it should. The white blood cells in your body attack healthy cells instead of bacteria and viruses. This is what causes inflammation in your blood vessels, but we don’t know exactly why this happens.

There is evidence to suggest pulmonary vasculitis is linked with:

• autoimmune disorders like arthritis or lupus
• infections like tuberculosis (TB), Legionnaires' and Pseudomonas
• drugs like penicillin
• conditions like COPD and asthma
• genetics – some children get pulmonary vasculitis from genes passed on from their biological parents.

There are lots of different types of vasculitis. They are usually grouped by the size of the blood vessels that are affected.

Small vessel vasculitis affects your capillaries and arterioles. These small vessels carry blood to your organs. Types of vasculitis that affect small vessels include:

• Granulomatosis with Polyangiitis (GPA) – this used to be called Wegener's granulomatosis. It can affect your lungs, but can also affect your ears, nose, sinuses and kidneys. It’s most common in older adults
• Microscopic Polyangiitis (MPA) – this mainly affects your lungs and kidneys but can also affect your skin and nerves. It’s most common in older adults
• Eosinophilic Granulomatosis with Polyangiitis (EGPA) – this used to be called Churg-Strauss syndrome. It usually affects your lungs and sinuses but can also affect your heart, kidneys, nerves and skin. It’s most common in adults. Most people with EGPA also have asthma.
• Anti-GBM disease (Goodpasture's disease) – this usually affects the lungs and kidneys of people aged 20 to 30, as well as those in their 60s and 70s.

Medium vessel vasculitis affects medium-sized arteries like the pulmonary artery which carries blood to your lungs.

• Kawasaki disease – this usually affects children under the age of five.

Large vessel vasculitis affects the aorta and its main branches. This is the main artery that carries blood away from your heart. Types of large vessel vasculitis include:

• Takayasu's arteritis – this usually affects women under the age of 40
• Behçet's disease – this usually affects children and people aged 20 to 40.

Vasculitis UK has more information about the different types of vasculitis.

Symptoms of pulmonary vasculitis vary because it can affect different parts of the body.

Symptoms affecting the lungs may include:

• coughing
• coughing up blood (damaged blood vessels can cause bleeding)
• finding it hard to breathe
• chest pain.

You may also have more general symptoms, such as:

• weight loss
• loss of appetite
• fever
• tiredness
• a rash, if the vasculitis affects your skin
• blood in your urine, if the vasculitis affects your kidneys.

If your healthcare professional thinks you or your child has pulmonary vasculitis, you will have blood tests and a urine test. You may also need a few other tests to look at your lungs in more detail:

• an X-ray or CT scan – to 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 the lungs
• lung biopsy – a surgeon removes some tissue from the lung. This can sometimes be done using keyhole surgery. If your kidneys might be affected, you may also have a kidney (renal) biopsy.

It can take time to get the right diagnosis because the symptoms of pulmonary vasculitis can be similar to other conditions.

Pulmonary vasculitis is a long-term condition. Although there are treatments to improve symptoms, there is no cure at the moment. Once you or your child have been diagnosed, you’ll be treated by specialists.

It’s important to get treatment as soon as possible. This can help to prevent or limit long-term damage to your lungs or other organs. Treatment includes:

• steroid therapy – corticosteroids (also known as steroids) will help to reduce the inflammation
• medicine to suppress (calm) the immune system – there are many different types of medicine that could be used, like biologic therapies.  Treatment will depend on how severe your condition is and what might have caused it.

Your specialist will talk to you in detail about the treatment before starting it. They can also tell you about clinical trials and any new treatments.

Living with pulmonary vasculitis is different for everyone and depends on how severe your condition is, and whether any organs are damaged.

Many people respond well to medicines. Others might have more severe disease and will need to take long-term medication.

Ask your healthcare professional who to contact if you have any questions, or if you’re unsure about something. It’s a good idea to agree a plan with your specialist about what you should do if you, or your child, become more unwell. You should also talk to your specialist about when you should seek emergency care.