Systemic Vasculitis

Systemic vasculitis is a group of rare autoimmune disorders characterised by inflammation of blood vessels. It can damage blood vessels and keep them from carrying blood to body parts that need it.

There are many types of systemic vasculitis, which can affect many different parts of the body. It can range from mild to severe, or even life-threatening. Some examples include:

• Takayasu arteritis
• Giant cell arteritis
• Polyarteritis nodosa
• Granulomatosis with polyangiitis
• Microscopic angiitis
• Eosinophilic granulomatosis with polyangiitis
• Henoch-Schönlein pupura
• Behçet’s disease

Vasculitis can also happen in people with another disease such as rheumatoid arthritis or systemic lupus erythematosus.

Symptoms of systemic vasculitis vary from one person to another and depend upon the type of vasculitis and location in the body of the inflamed blood vessels.

Some common symptoms include:

• Fatigue or weakness
• Fever
• Lack of appetite and weight loss
• Rash
• Persistent headaches, vision loss
• Pain in the jaw
• Nerve problems (numbness, weakness, pain)
• Nose bleeds, difficulty with smells
• Cough, blood in phlegm, breathlessness
• Frothy urine
• Abdominal pain after meals
• Joint pain, muscle aches
• Chest pain when walking fast
• Fingertips turning blue in the cold

In most cases, the cause is unknown. A combination of factors may put a person at risk of developing systemic vasculitis:

• Other autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus may be risk factors
• Systemic vasculitis can develop as a reaction to certain drugs
• Systemic vasculitis can also develop as a result of infection such as hepatitis B, hepatitis C, human immunodeficiency virus or some infections caused by bacteria

It can be difficult to diagnose systemic vasculitis as the symptoms are similar to those caused by a number of other illnesses. Your doctor will speak to you, perform a physical examination, and may order tests to help establish a diagnosis.

The tests used to diagnose systemic vasculitis depend on the type of systemic vasculitis that is suspected and may include the following:

• Blood tests such as full blood count, kidney function and auto-antibodies
• Urine tests
• Imaging tests such as X-rays, computer tomography scans or magnetic resonance imaging (MRI)
• Nerve testing
• Lung function testing
• Biopsy of skin, kidney or nasal cavity tissues or other locations affected by vasculitis. A biopsy is often required to be sure of the diagnosis before starting treatment.

Some of the symptoms of systemic vasculitis are also frequently seen among older people with cancers. Therefore, during the process of diagnosis for systemic vasculitis, the doctor may also speak to you about screening tests for cancers.

The treatment of systemic vasculitis will depend on the specific type of vasculitis and the areas of the body that are involved.

Treatment may include one or more of the following:

• Medications

• Glucocorticoids (also called “steroids”). High doses of glucocorticoids may be given into a vein, or oral tablets such as prednisolone may be given. Some people require treatment with long-term glucocorticoids to control symptoms and prevent worsening of the systemic vasculitis. Close monitoring for side-effects of glucocorticoids, such as weight gain, diabetes, osteoporosis (bone-thinning) is needed. When the condition improves, the dosage is then slowly reduced.

• Additional treatment that suppresses the immune system (i.e. immunosuppressive medications) are also needed for some types of systemic vasculitis. The choice of treatment depends on the specific type of vasculitis and the severity of the disease. Some examples of medications that may be used include:

• Severe types of vasculitis: Cyclophosphamide, rituximab and plasma exchange.
• Less severe types of vasculitis: Azathioprine, methotrexate, mycophenolate. These drugs may also be used as “maintenance therapy”, i.e. to control the disease after initial treatment.

All immunosuppressive medications have the potential of increasing the risk of infections, and each type of medication has important side effects that must be monitored for by a doctor.


• General Measures