The term vasculitis refers to a rare group of diseases that are characterised by the presence of inflammation in the blood vessels. Vasculitis is a condition that can occur on its own (primary vasculitis), or in connection with another illness such as rheumatoid arthritis or systemic lupus erythematosus (secondary vasculitis). The severity of the illness can vary greatly from very mild, where no specific therapy is required, to severe life threatening multi-system diseases which need specialist hospital care. A primary vasculitic illness that involves many different organ systems in the body may also be referred to as 'systemic vasculitis'. Within the umbrella of this term are several sub groups of vasculitis which are classified on the basis of the size of the blood vessels that are inflammed or specific clinical features. Classification in this way allows greater understanding of the mechanisms involved in disease pathogenesis, the prognosis and optimum treatment for each individual.

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

Giant cell arteritis		Takayasu arteritis
Polyarteritis nodosa		Kawasaki disease
Wegeners Granulomatosis		Churg-Strauss Syndrome
Microscopic polyangiitis		Henoch-Schonlein purpura
Essential cryoglobulinaemic vasculitis		Behcets disease
Cutaneous leukocytoclastic vasculitis

However, in all types of vasculitis, the basic problem is that there is inflammation in the blood vessel wall. White blood cells infiltrate the vessel wall leading to damage of the surrounding tissue and obstruction of the vessel itself. Blood supply to vital tissues may then be impaired, leading to a potentially damaging effect on the organ which is being supplied. The severity of the illness generally depends on the site, size and extent of vessel involvement.

In more generalised disease several internal organs may be involved (e.g. the lungs and kidneys). The spectrum of organ involvement will determine how ill the patient feels as well as the therapy that is needed and how quickly it should be started.

A diagnosis of a vasculitic illness is often made on clinical features (medical history and physical examination), with supporting information from specific tests (blood tests, X-rays and tissue biopsy samples). Because of the complexity and multi-system of the nature of these diseases it is often necessary to involve doctors from many different specialities in management decisions.

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 people 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 rate of relapse and 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 or drug therapy. There is no doubt that something causes the body’s defenses to start working against itself, instead of just the intruders. But in most people it can occur by itself where no cause has as yet been found.

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Symptoms and What to Do About Them:

PLEASE REMEMBER - as part of the illness symptoms come and go and it is better to seek advice early and get appropriate treatment, in order to prevent damage caused by new areas of inflammation. However, your doctor will decide whether a new symptom has anything at all to do with your vasculitis.

When the disease first appears, symptoms may include unexplained weight loss, unexplained fever, skin lesions, muscle pain, joint pains, and mouth ulcers or genital ulcers that do not go away. Some symptoms are associated more with a particular form of vasculitis than others, such as sinus problems with Wegeners Granulomatosis and genital ulcers with Behcets disease. As you become more aware of how your vasculitis effects you as an individual, then you appreciate that worsening of these symptoms may be a sign of a flare.

Also, many people with vasculitis report a feeling of immense fatigue. Everybody feels tired occasionally, but this fatigue may be severe. It is this aspect of the disease that many patients find very hard to cope with. Added to this, it is a symptom that cannot be seen and is easily misunderstood. Knowing this is common, and accepting it, can be helpful to you and your family.

Included in this section is a list of symptoms that may be connected with vasculitis. No one will have them all, and many will have just a few depending on which part of the body is affected. However, some symptoms may be easily ignored, for example, sinus problems, and a trip to the doctor is delayed. For some patients this is an important feature but for others it’s not. When the illness is new to you, it is advisable to let the doctor know what is happening and let him decide what is important or not. However, as you learn more about your disease and your own body, you will be able to decide what needs reporting. But if in doubt, do contact your doctor.[Back]

Review of Symptoms
When you should see a doctor

Some people with vasculitis report that they always “look well” even when they are feeling at their worst! (This may be a side effect of taking prednisolone, or just because the disease itself can be such a hidden one). If you know this applies to you, it would be helpful to remind your doctor of this whenever you are consulting him regarding new or ongoing medical problems. The severity of your symptoms may be underestimated if your doctor is unaware that how you look is not necessarily an accurate indicator of your real state of health. [Back]