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Vasculitis Information

Vasculitis can occur as the main disease (primary vasculitis), or as a complication of another disease (secondary vasculitis). It can just affect one part of the body (localized vasculitis) or multiple parts of the body (systemic vasculitis).

There are almost 20 types of 'primary systemic vasculitis' and they are classified as to whether they mainly affect small, medium sized or large blood vessels. Within the small vessel subgrouping the most common forms of Vasculitis are associated with an ANCA blood test (antineutrophil cytoplasmic antibodies) and this subgroup comprises Granulomatosis with Polyangiitis (formerly Wegener’s Granulomatosis), Microscopic Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis (formerly Churg-Strauss). The next most common types are those that affect large blood vessels, Giant Cell (temporal) Art

eritis and Takayasu's Arteritis.

Individual variants of vasculitis are defined by the parts of the body affected (for example, Granulomatosis with Polyangiitis typically affects the nose, lungs and kidneys), by features seen on tissue biopsy and by the pattern of blood test abnormalities. Direct imaging of blood vessels by X-rays or other tests helps in the diagnosis of medium and large vessel Vasculitis.


Who gets it?

We don't really know.

Different variants affect different age groups, ANCA associated vasculitis is most common in those over 50 years but can affect younger patients, while Kawasaki disease mainly affects young children and Takayasu's Arteritis, young adults. Giant Cell Arteritis is most common in those over 70 years old.

Geographic regions affect the types of Vasculitis seen, with Granulomatosis with Polyangiitis (Wegener’s) and Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss) being more common in colder climates. Ethnicity is also a factor with Chinese and Japanese populations having more Microscopic Polyangiitis and Takayasu's Arteritis and less Granulomatosis with Polyangiitis (Wegener’s) and Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss).

The frequency of secondary Vasculitis depends on the underlying disorder and chronic viral hepatitis infection is probably the most common cause.


Is it hereditary or family tendency?

Not really.

There is a complex genetic predisposition which is not fully understood. Family members only have a slightly increased risk of developing Vasculitis when compared to the general population, so it is not regarded as an inherited disease.


What causes it?

A consensus view is that an environmental agent, such as a virus, triggers the disease in a genetically susceptible individual.

Environmental exposure to silica in coal mining increases the likelihood of Vasculitis but other environmental factors have not been identified. Infections, such as with the bacteria, staphylococcus and sterptococcus, have been implicated.


How would you know you had it?

This varies between the variants of Vasculitis.

Typically there is a period of time (prodromal phase) of fluctuating symptoms including tiredness, muscle aches, joint pains, headaches, fevers, sweats and weight loss.

Then there are features of a particular part of the body being affected. This is obvious if it is a skin rash. Granulomatosis with Polyangiitis (Wegener’s) and Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss) affect the nose, sinuses and ears with nasal stuffiness, bleeding, sinus pain and deafness. Lung involvement could cause cough, wheeze, coughing blood or breathlessness. Kidney involvement causes no obvious symptoms until the kidneys are badly damaged, but can be detected at an early stage by a simple urine 'dip stick' test.


How would it be diagnosed?

The key is suspicion. Vasculitis can be hard to diagnose.

But it should be suspected in patients with an unexplained illness where there is evidence of 'inflammation' on the ESR or CRP blood tests.

Then there are many specific scenarios when it should be suspected which depend on the part of the body affected. For example, it should be suspected in anyone with a urine test suggesting kidney disease or in a patient with unexplained shadows on a chest X-ray or the development of asthma in adulthood.

Once it has been suspected, the doctor should launch quite a wide range of investigations that will lead to confirmation of Vasculitis or the discovery of an alternative disease.


Can it be treated?

Yes. Treatment is quite complicated and its success depends on the type of Vasculitis, the parts of body affected and the severity but Vasculitis can usually be controlled. Quite often the Vasculitis will have already caused damage that cannot be reversed though, for example change in shape of the nose, but treatment should stop further damage occurring. Most patients will feel very unwell when their Vasculitis is untreated and it can take many months for them to feel stronger after treatment is started.

Does it ever go away?

In some variants.

Again this depends on the type. Some forms of Vasculitis only occur once and do not recur, for example Henoch-Schonlein Purpura in children. Other types are more likely to remain present but controlled through treatment. The disease may flare as treatment is reduced or withdrawn.


Are you at risk of any other illnesses?

If a part of the body is damaged, for example the kidneys, then a patient will suffer the consequences of kidney failure. Treatment is associated with a number of early and late side-effects and make patients susceptible to infections. Over the long term there is a slightly increased risk of cardiovascular disease (heart disease and stroke) and of cancer.


Is it likely to shorten your life?

This depends on the type of Vasculitis, its severity and whether damage has occurred. Damage to the kidney is the most common cause of a shortened life span. Very severe Vasculitis presentations can be fatal.

Who should you see for help?

Vasculitis patients should be treated by doctors experienced in the disease. Vasculitis doctors are most commonly trained as rheumatologists (joint doctors) or nephrologists (kidney doctors) but other specialists may have developed an interest and experience in the disease. There is no system within the NHS in the UK for either training Vasculitis doctors or of Vasculitis clinics, but many hospitals have developed there own Vasculitis clinics.

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