Vasculitis

 

What is Vasculitis?

Vasculitis means inflammation in the blood vessels. It is a broad name for a group of conditions that affect different sizes of blood vessel.

 

Blood vessels transport blood around the body – arteries take blood from the heart to the organs and veins transport blood back to the heart. Arteries and veins around the heart start off large, and as they move further away, become smaller. The smallest vessels are called capillaries; these pass oxygen and nutrients to muscles and organs.

 

The immune system usually produces inflammation in response to injury or infection.  However, in vasculitis, inflammation is targeted at healthy blood vessels, causing damage to the vessel wall which narrows the channel and leads to reduced blood supply to tissues.  This can result in thinning and ballooning of the walls causing aneurysms. The overactivation of the immune system against normal blood vessels suggests that most forms of vasculitis are autoimmune diseases.

 

There are many types of vasculitis; the easiest way to classify them is by the size of the blood vessel they affect.

 

Subtypes of vasculitis

Large arteries

Medium arteries

Small vessels and medium arteries

Small vessels

 

Causes of vasculitis

The cause of vasculitis is unknown, but evidence suggests it is caused by a number of factors such as genetics and environmental factors.

Some genes have been linked to increased risk of developing vasculitis, although it isn’t directly inherited and other triggers are likely needed.

There are some well-known environmental triggers for vasculitis. In these cases, we would call these secondary vasculitides.  Viral and bacterial infections in particular are known to cause certain types of vasculitis. Smoking and UV light exposure are also implicated. Certain medications are also linked to developing vasculitis, these usually only cause skin rashes and resolve on withdrawing of the drug.

Rarely a small number of cancers have been known to cause vasculitis.

 

Symptoms of vasculitis

Different types of vasculitis often present in different ways, however there are some common symptoms of all:

General symptoms

 

Specific areas can be involved in certain types of vasculitis:

 

Skin lesions

Eyes

Lung and airway involvement

Heart involvement

Kidney involvement

Gut involvement

Nervous system

 

How do we diagnose vasculitis?

Diagnosis is made based on history, examination and investigations.

 

Blood tests:

Urine dip- to check for protein and blood

Imaging

Heart investigations

Nerve tests

Biopsies

 

Types of Vasculitis

Giant Cell Arteritis (GCA)

Takayasu Arteritis

Polyarteritis Nodosa (PAN)

ANCA associated vasculitis

IgA Vasculitis

Cryoglobulinaemia

 

Treatment 

Management of vasculitis is tailored to the type of vasculitis and the blood vessels involved. The aim of treatment is to prevent any complications and permanent damage to organs such as the heart, kidneys and brain.

Treatment is based on quickly inducing remission (control) of the inflammation and then maintenance treatments are used to prevent any further flares of the disease.

 

Remission induction is based on the use of high dose steroids and in some severe life threatening cases of vasculitis, other medications to suppress the immune system are used such as DMARDs and biological therapies.

 

Maintenance therapies include long-term use of DMARDs.

In some patients, once they have been stable with no flares of the disease, treatment can be slowly withdrawn.

 

If a clear cause is found to have caused the vasculitis such as a hepatitis infection or drug, then management of these is important.

 

Rarely if the damage to the blood vessels is severe causing aneurysms, surgery may be indicated.

 

Useful links

Versus Arthritis: http://www.versusarthritis.org

Vasculitis Scotland Support network: http://vasculitis-scotland.org

Vasculitis UK: https://www.vasculitis.org.uk