Vasculitis Disorders

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Vasculitis disorders result from inflamed of blood vessels. They are relatively rare and can affect people of all ages. Granulomatosis with polyangiitis (also known as Wegener’s granulomatosis) is the most common form of vasculitis, and it affects around five in a million people.

Vasculitis disorders cause a wide range of symptoms, that can affect the skin and internal organs. Treatment length varies, and some people need to use medications for long periods of time.

What is vasculitis?

Vasculitis disorders result from inflammation of blood vessels, including arteries, arterioles, veins, venules and capillaries. The inflammation causes a narrowing of blood vessels, which can result in blood flow obstruction (ischaemia).  This may lead to tissue damage (necrosis) and blood clots (thrombosis). 

What causes vasculitis?

There are three main underlying causes of vasculitis disorders:

Allergy, hypersensitivity and infections should be ruled out before autoimmunity is considered as the cause of a vasculitis disorder.  

Autoimmune related vasculitis

The main role of the immune system is to defend against infections (such as bacteria, moulds and viruses) and other invaders (such as cancer cells), whilst protecting the body’s own cells. Autoimmunity occurs when the body doesn’t recognise its own cells and attacks them.

Antibodies produced by the immune system in vasculitis disorders cause inflammation in blood vessels that can lead to problems. Complications depend on which blood vessels, organs and other systems are affected.   

Vasculitis disorders may also occur in people with other autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus (SLE) and dermatomyositis. 

Diagnosis 

Due to the wide range of signs, symptoms and body systems involved, an extensive history and physical examination is needed to diagnose the type of vasculitis disorder. Blood tests are taken, and in some cases an x-ray or biopsy may be required. An exact diagnosis is needed to provide the right treatment.

Treatment

When vasculitis is due to an autoimmune disorder, immunosuppressive drugs are usually used.

Plasmapheresis (a procedure that filters the offending autoantibodies out of the blood plasma and returns the filtered blood back to the patient), may be used in serious cases that do not respond to other treatment.

Signs and symptoms

General signs and symptoms of vasculitis disorders include:

Specific signs and symptoms of vasculitis disorders include:

Examples of vasculitis disorders (small blood vessels)

Examples of vasculitis disorders (medium blood vessels)

Examples of vasculitis disorders (large blood vessels)

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Updated May 2019