Vasculitis / Capillaritis

What is Vasculitis and Capillaritis?

Vasculitis happens when there is inflammation of blood vessels. This can happen anywhere in the body. When this happens in the lungs it is called pulmonary vasculitis. Usually pulmonary vasculitis is caused by an inflammatory disease of the whole body. These diseases include: granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and Goodpasture’s disease.

Pulmonary capillaritis happens when there is inflammation of the very tiny blood vessels of the lung. These blood vessels surround the alveoli (lung air sacs). This can happen with whole body inflammatory disease. It can also happen alone without other body systems being involved.

Both vasculitis and capillaritis can cause bleeding into the air spaces of the lungs. Children may have cough and trouble breathing. Some children will cough up blood or bloody mucus. They may also have low oxygen levels.

Watch: Vasculitis — Vasculitis Foundation

Diagnosis

Vasculitis or capillaritis is possible when children have trouble breathing and low red blood cell counts (anemia).

• A chest x-ray may show areas of pneumonia.
• A lung scan (chest CT) may show many areas of pneumonia or fluid in the lungs.
• A lung scope (bronchoscopy) can show normal airways. The lung wash (lavage) will show signs of blood deeper in the lungs. The fluid may look bloody during the scope. It may also show blood when doctors look at it using a microscope.
• A complete blood count (CBC) may show low red blood cell counts (anemia).
• Blood tests may show signs of body system inflammation or antibodies causing inflammation.
• A lung biopsy is sometimes needed for the right diagnosis. This will show blood and inflammation in the air spaces and blood vessels.

Treatment

Doctors need to treat this with strong medicines to block inflammation in the lungs. Without treatment, the inflammation can cause serious lung damage. These medicines include high dose steroids and other medicines like cyclophosphamide or rituximab. Sometimes doctors need to “clean the blood” and get rid of antibodies causing inflammation. This is called plasmapheresis. Children usually need medicines for several months. Your medical team will help decide which medicines to use and how long to use them.

Treatment is also to support breathing. Children may need oxygen to help keep their oxygen levels normal. They may need positive pressure to help them breathe oxygen in and carbon dioxide out. Children who are very sick may need to be in the intensive care unit for help breathing.

What Does Vasculitis and Capillaritis Mean for My Child?

Vasculitis and capillaritis can cause different levels of illness in children. Some children only have mild bleeding in the lungs. Some children have very bad bleeding that can make them very sick. Some children can die from lung bleeding. It is important to start treatment early and support breathing. Children usually need to stay on medicines for a long time to keep from bleeding.

It is important to protect your child’s lungs from infection by making sure they get the recommended vaccines.

What to Watch Out For

• If your child is working hard to breathe, talk to a health care provider. If possible, ask to see a lung specialist who is experienced with these conditions.
• If your child needs to wear oxygen, work with your health provider to get the correct amount to give your child. This amount may change from time to time.
• If your child struggles with feeding or gaining weight, talk to your health care provider.
• Try to prevent infections from common childhood illnesses. Wash your hands often and ask your friends and family to let you know if they are sick before they are around your child.
• Get your child’s vaccines and yearly flu shot.

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This information is for educational purposes only. It should not be used as a substitute for the medical advice of one’s healthcare provider.