Vasculitis

Overview

Vasculitis means inflammation of the blood vessels.

Inflammation is your immune system's natural response to injury or infection. It causes swelling and can help the body deal with invading germs.

But in vasculitis, for some reason the immune system attacks healthy blood vessels, causing them to become swollen and narrow.

This may be triggered by an infection or a medicine, although often the cause is unknown.

Vasculitis can range from a minor problem that just affects the skin, to a more serious illness that causes problems with organs like the heart or kidneys.

There are many types of vasculitis. The rest of this page discusses a range of potential causes.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Eosinophilic granulomatosis with polyangiitis, also called Churg-Strauss syndrome, is a type of vasculitis that mainly affects adults aged 30 to 45.

It can cause:

It can also affect the nerves, causing weakness, pins and needles or numbness, and sometimes damages the kidneys or heart muscle.

It's usually treated with steroid medicine.

The Vasculitis UK website has more information about eosinophilic granulomatosis with polyangiitis.

Giant cell arteritis (temporal arteritis)

Giant cell arteritis is a type of vasculitis that often affects the arteries in the head and neck. 

It mostly occurs in adults over the age of 50.

It's sometimes called temporal arteritis because the arteries around the temples are often affected.

It can cause:

  • aching and soreness around the temples
  • jaw muscle pain while eating
  • headaches
  • double vision or vision loss

It also commonly occurs alongside polymyalgia rheumatica.

The main treatment is steroid medicine.

Granulomatosis with polyangiitis (Wegener's granulomatosis)

Granulomatosis with polyangiitis, also called Wegener's granulomatosis, is a type of vasculitis that mainly affects blood vessels in the nose, sinuses, ears, lungs and kidneys.

It mainly affects middle-aged or elderly people.

It can cause:

It's a serious condition that can be fatal if left untreated, as it can lead to organ failure.

It's usually treated with steroid medicine or other medicines that reduce the activity of the immune system.

Henoch-Schönlein purpura

Henoch-Schönlein purpura is a rare type of vasculitis that usually occurs in children and can affect the skin, kidneys or bowel.

It's thought to be triggered by the body reacting to an infection.

It can cause:

It's not usually serious and tends to get better without treatment.

Kawasaki disease

Kawasaki disease is a rare condition that can occur in children under the age of 5. 

The characteristic symptoms are a high temperature that lasts for 5 days or more, with:

  • a rash
  • swollen glands in the neck
  • dry, cracked lips
  • red fingers or toes
  • red eyes

Intravenous immunoglobulin (IVIG), a solution of antibodies, and aspirin are the 2 main medicines used to treat Kawasaki disease.

Aspirin is not usually recommended for children under 16, so do not give aspirin to your child unless advised to by their doctor.

Microscopic polyangiitis

Microscopic polyangiitis is a rare and potentially serious long-term type of vasculitis that most often develops in middle-aged people.

It can affect any organ, but particularly affects the lungs, kidneys and nerves.

It can cause:

It's usually treated with steroid medicine or other medicines that reduce the activity of the immune system.

The Vasculitis UK website has more information about microscopic polyangiitis.

Polyarteritis nodosa

Polyarteritis nodosa is a rare type of vasculitis that particularly affects the arteries supplying the gut, kidneys and nerves.

It tends to develop in childhood or in middle-aged people.

It can sometimes be triggered by an infection, such as hepatitis B, but the exact cause is uncertain.

It can cause:

It can be very serious if not treated. The main treatment is steroid medicine, and sometimes other medicines that reduce the activity of the immune system.

The Vasculitis UK website has more information about polyarteritis nodosa.

Polymyalgia rheumatica

Polymyalgia rheumatica is a type of vasculitis that's closely related to giant cell arteritis. 

It mostly occurs in adults over 50 and is more common in women than men.

It can cause:

  • pain and stiffness in the shoulders, neck and hips, which is often worse after waking up
  • a high temperature
  • extreme tiredness
  • loss of appetite and weight loss
  • depression

The main treatment is steroid medicine, which is usually used in lower doses than for giant cell arteritis.

Takayasu arteritis

Takayasu arteritis is a type of vasculitis that mainly affects young women. It's very rare in the UK.

It affects the main artery from the heart, as well as the major arteries branching off it.

It can cause:

Treatment is usually with steroid medicine.

The Vasculitis UK website has more information about Takayasu arteritis.

Other types of vasculitis

Behçet's disease

Behçet's disease typically causes mouth ulcers and genital ulcers, and is more common in people from Greece, Turkey, the Middle East, China and Japan.

Buerger's disease

Buerger's disease affects blood vessels in the legs and arms, leading to reduced blood flow to the hands and feet. It's closely linked to smoking.

Cogan's syndrome

Cogan's syndrome is inflammation of the blood vessels in the inner ears and eyes.

Cryoglobulin-associated vasculitis

Cryoglobulin-associated vasculitis is associated with proteins in the blood called cryoglobulins and can occur after a hepatitis C infection.

It causes a rash on the lower limbs, joint pain, nerve damage, tummy (abdominal) pain and kidney problems

Hypersensitivity vasculitis

Hypersensitivity vasculitis is usually caused by a reaction to a medicine, such as non-steroidal anti-inflammatory drugs (NSAIDs) or certain antibiotics, and results in a temporary rash.

Primary angiitis of the central nervous system

Primary angiitis of the central nervous system is inflammation of the blood vessels in the brain

Rheumatoid vasculitis

Rheumatoid vasculitis is vasculitis associated with rheumatoid arthritis



The information on this page has been adapted by NHS Wales from original content supplied by NHS UK NHS website nhs.uk
Last Updated: 02/12/2022 13:48:43