What is vasculitis?

Vasculitis is a relatively rare disease that causes inflammation of blood vessels. It is an autoimmune disease, which means the immune system attacks the body's own cells, tissues and organs. It is also known as angiitis and arteritis.

The inflammation can cause blood vessels to narrow. This may restrict blood flow to part of the body (ischaemia) or stop it altogether (thrombosis). Sometimes the vessel wall may weaken and balloon out (an aneurysm), which can cause bleeding.

Inflammation can also lead to tissue damage affecting one or many body organs such as the eyes, skin, lungs, gut, kidneys or nervous system.

What are the types of vasculitis?

There are a number of types of vasculitis, including:

• Granulomatosis with polyangiitis
• Eosinophilic granulomatosis
• Cryoglobulinaemia
• Goodpasture's syndrome
• Henoch-Schonlein purpura
• Microscopic polyangitis
• Behcet’s disease
• Central nervous system vasculitis
• Kawasaki syndrome
• Polyarteritis nodosa
• giant cell (temporal) arteritis
• Takayasu arteritis
• Polymyalgia rheumatic

What are the symptoms of vasculitis?

The symptoms of vasculitis can vary greatly, depending where in the body the inflammation is. Some signs that may be seen in all types of vasculitis include:

• feeling very tired
• fever
• muscle or joint pain
• poor appetite
• weight loss
• numbness or weakness in various parts of the body

Other symptoms might develop, depending on the type of vasculitis.

• Skin: rash, sores or purple or red spots, urticaria, itching
• Joints: aches and pains, arthritis
• Lungs: shortness of breath, pneumonia, coughing up blood
• Eyes: blurred vision, sensitivity to light, redness, itch and burning
• Digestive tract: mouth ulcers, abdominal pain
• Sinuses, nose, throat and ears: stuffy or bloody nose, repeated ear infections, ulcers in the nose, hearing loss
• Brain: headache, changes in mental function, muscle weakness, paralysis
• Nerves: numbness, tingling, weakness, shooting pains

What causes vasculitis?

There are 3 main causes of vasculitis:

• an autoimmune reaction, where the immune cells attack the body. Vasculitis can happen in people with other autoimmune diseases such as rheumatoid arthritis, lupus or dermatomyositis
• an allergy or hypersensitivity to medications, toxins or other substances that are inhaled
• an infection from a virus or parasite

When should I see my doctor?

It is important to seek help right away if you think you have vasculitis, as early treatment may help prevent long-term damage.

How is vasculitis diagnosed?

It can be difficult to diagnose vasculitis because the symptoms can be similar to those caused by some other illnesses.

Your doctor will ask you about your symptoms and examine you.

You may have blood tests and urine tests, and imaging tests such as an x-ray (including angiography — a special type of x-ray that looks at the blood vessels), CT scan or MRI scan. In some cases, lung function tests or nerve conduction studies may also be done.

To confirm a diagnosis of vasculitis, a small sample of tissue (a biopsy) is often needed to examine under the microscope.

How is vasculitis treated?

If the vasculitis is caused by an allergy or hypersensitivity, removing the substance usually stops the symptoms.

If it is due to an autoimmune condition, treatment can help control the symptoms and often leads to remission of the vasculitis (where symptoms become less severe or disappear). Some people have chronic (long-term) vasculitis, however, and never go into remission.

Medicines for vasculitis work by:

• controlling the inflammation (for example, corticosteroids)
• suppressing the immune system (for example cyclophosphamide, methotrexate or azathioprine)

Newer medications called ‘biologics’ are increasingly being used to treat some forms of vasculitis.

Vasculitis complications

The medicines used to manage vasculitis often have to be taken for a long time and can have side effects. It is important to talk to your doctor about your medicines and their advantages and disadvantages.

Eating well, exercising and stopping smoking if you smoke can help to minimise side effects.

Resources and support

Vasculitis is often a chronic disease and you may need emotional support from your family and friends to help you cope.

Your doctor may also be able to provide you with information about support groups for people with vasculitis.

The Autoimmune Resource & Research Centre can provide support and information.