About Vasculitis

Vasculitis is an auto-immune disease which causes inflammation of the blood vessels. It can occur as the main disease (‘primary vasculitis’), or as a complication of another disease (‘secondary vasculitis’). It can 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’. They are classified by whether they mainly affect small, medium sized or large blood vessels.

Within the small vessel type, the most common forms of vasculitis are associated with an ANCA blood test (antineutrophil cytoplasmic antibodies). This subgroup includes Granulomatosis with Polyangiitis (formerly Wegener’s Granulomatosis), Microscopic Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis (formerly known as Churg-Strauss).

The next most common types are those that affect large blood vessels, and include Giant Cell (temporal) Arteritis 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.

We have factsheets with more information on each type of vasculitis, as well as other topics you may find helpful.

SEE OUR VASCULITIS FACTSHEETS

Some of the different types of vasculitis are:

• Behcets Disease
• Buerger’s Disease
• Central Nervous System Vasculitis
• Cryoglobulinaemia
• Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly known as Churg-Strauss)
• Giant Cell Artertitis
• Granulomatosis with Polyangiitis (GPA, formerly known as Wegener’s)
• Henoch Schonlein Vasculitis (HSP)
• Kawasaki Disease
• Micropscopic Polyangiitis MPA
• Polyarteritis Nodosa
• Polymyalgia Rheumatica
• Rheumatoid Vasculitis
• Takayasu Arteritis

We have factsheets with more information on each type of vasculitis, as well as other topics you may find helpful.

SEE OUR VASCULITIS FACTSHEETS

The short answer is: anyone. There’s not a specific ‘type’ of person who gets vasculitis.

Different variants of vasculitis tend to affect different age groups. For example, ANCA associated vasculitis is most common in those over 50 years, but it can also affect younger patients. 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 can also affect the types of vasculitis seen. Granulomatosis with Polyangiitis (Wegener’s) and Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss) are more common in colder climates.

Ethnicity is also a factor. For example, Chinese and Japanese populations have more Microscopic Polyangiitis and Takayasu’s Arteritis, and fewer instances of 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.

We don’t know yet – there’s a complex genetic predisposition which is not fully understood. But family members have only a slightly increased risk of developing vasculitis when compared to the general population, so vasculitis is not regarded as an inherited disease.

There’s a consensus that an environmental agent, such as a virus, triggers vasculitis in a genetically susceptible individual.

There’s evidence that 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 also been implicated.

Symptoms vary between the different variants of vasculitis. But typically, there’s a period of time (called the ‘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. The nose, sinuses and ears can be affected by Granulomatosis with Polyangiitis (Wegener’s) and Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss) with symptoms including nasal stuffiness, bleeding, sinus pain and deafness.

Lung involvement could cause coughing, wheezing, 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.

Vasculitis can be hard to diagnose – but the key is suspicion. If you haven’t had a diagnosis of vasculitis but think you might have it, it’s important that you speak to your doctor about it. Once it has been suspected, your doctor should launch quite a wide range of investigations that will lead to either confirmation of vasculitis or an alternative diagnosis.

Scenarios where vasculitis could be suspected could include:

• When a patient with an unexplained illness shows evidence of ‘inflammation’ on ESR or CRP blood tests.
• In specific scenarios depending on which part of the body is affected. For example,
  • with a urine test suggesting kidney disease
  • in a patient with unexplained shadows on a chest X-ray
  • with the development of asthma in adulthood.

Once you have a diagnosis, LCTF can help with practical and emotional support.

GET SUPPORT

Yes, vasculitis can be treated. 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.

It’s not unusual, though, for the vasculitis to have caused damage that cannot be reversed – for example, changing the shape of your 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.

We have factsheets with information on different treatments for vasculitis, which you may find helpful.

SEE OUR VASCULITIS FACTSHEETS

If vasculitis goes away or not will depend on the type of vasculitis. Some forms of vasculitis only occur once and don’t 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.

There’s a chance that you could develop other conditions or illnesses, either as a result of the vasculitis or because of the treatment for it.

If a part of the body is damaged by vasculitis – for example, the kidneys – then a patient will suffer the consequences of kidney failure.

Treatment for vasculitis is associated with a number of side-effects and can 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.

It is possible that vasculitis could shorten your life span. However, it depends on the type of vasculitis, its severity and whether damage has occurred. Damage to the kidneys is the most common cause of a shortened life span. Very severe vasculitis presentations can be fatal.

For information about your particular circumstances, it’s important that you discuss this with your doctor.

Once you have a diagnosis, LCTF can help with practical and emotional support.

GET SUPPORT

If you’re not already receiving treatment, your first port of call would usually be your GP. You should then be referred to a specialist, as 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.

Once you have a diagnosis, LCTF can help with practical and emotional support.

GET SUPPORT