Vasculitis

Vasculitis is a very rare disease that can vary from a very mild variety that needs no treatment to one involving many organs (systemic disease), which means the person ends up in hospital very ill. It is a condition that can occur on its own, or in connection with another illness that is already present, for example, rheumatoid arthritis or lupus.

When vasculitis is the main illness, it may be referred to as 'primary systemic vasculitis'. This term is used as a umbrella to classify different types of vasculitides depending on presenting symptoms and the blood vessels involved. Therefore, it is as well for each individual patient to ask for specific information from their own consultant regarding their individual diagnosis.

Some of the names given to different types of primary systemic vasculitis are given below:

  • Giant cell arteritis
  • Polyarteritis nodosa
  • Wegner's Granulomatosis
  • Microscopic polyangiitis
  • Essential cryoglobulinaemic vasculitis
  • Cutaneous leukocytoclastic angiitis
  • Takayasu arteritis
  • Kawasaki disease
  • Churg-Strauss Syndrome
  • Henoch-Schonlein purpura
  • Behcet's disease

In all types of vasculitis, the basic problem is that a blood vessel has become inflamed. This leads to problems with the blood flow that can then interfere with the function of the organs supplied by that blood vessel.

How ill the person feels, what treatment will be needed and how quickly treatment must be started depends on:

  • Where the inflammation is?
  • How bad the inflammation is?
  • How many blood vessels are involved?
  • How many major organs, for example lungs and kidneys, are involved?

To make a diagnosis of vasculitis, the doctor will need to take a complete medical history, carry out a physical examination and arrange for some tests to be carried out (for example, blood tests and X-rays). The tests will vary from individual to individual depending on what symptoms they have and what their history reveals. It may seem very frustrating for the patient if they have to repeat their symptoms they have and what their history reveals. It may seem more than one doctor (which is often the case).

However, it is important to be patient because getting the diagnosis correct is vital and can be a complex process.

Sometimes the first diagnosis gets changed, as more information is collected and the advice and opinion of different doctors is sought. This is essential for a rare disease. It can be quite unsettling and again, frustrating for the patient. However, as the disease often overlaps with other forms of connective tissue diseases, it is important for the doctors to keep an open mind whilst they are deciding what the correct diagnosis should be.

The Progress and Nature of Vasculitis

The course of vasculitis has changed since it was first described many years ago. With the advent of better treatments, many people can live with vasculitis. As there is no cure at present, it is important to understand that it is a lifelong condition. Although you may have had one episode of disease (such as patients with Henoch-Schonlein purpura), it may present again. This is called a relapse (or flare). When your disease is quiet it is said to be in remission. The frequency of relapse and length of remission varies between different types of vasculitis, and your doctor takes this into consideration when prescribing a treatment regime (i.e. what medicines are needed when and for how long).

What causes vasculitis? On rare occasions it may be caused by an infection that is then promptly treated with antibiotics. Sometimes it is due to a reaction by the immune system whereby the body's defences start working against itself. But in some people it can occur by itself where no cause has as yet been found. This is an area of active research in several specialist centres throughout Europe and the United States of America. Improved understanding through research will lead to more specific treatment.

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