Vasculitis

Vasculitis is the name for a group of conditions that cause inflammation of the blood vessels. This makes the vessel walls swell and thicken, which can restrict blood flow and, over time, damage the organs and tissues they supply.

What are the symptoms of vasculitis?

Symptoms vary widely depending on which blood vessels and organs are affected and can range from mild to severe. Common symptoms include:

• feeling generally unwell, with tiredness, fever, or unexplained weight loss
• muscle and joint pain
• rashes, bumps, or discoloured patches on the skin
• numbness, tingling, or weakness, especially in the hands or feet
• headaches, or changes to your vision
• shortness of breath or a cough
• tummy (abdominal) pain
• dark or bloody urine, which can be a sign of kidney problems.

You can have kidney problems caused by vasculitis without noticing any symptoms, which is why urine tests are often used to check for this.

What are the causes of vasculitis and who is at risk?

In most cases, the exact cause isn't known. Vasculitis is generally an autoimmune condition, meaning your immune system mistakenly attacks your own healthy blood vessels. A combination of factors is thought to trigger this, including:

• an infection
• a reaction to certain medications
• another autoimmune condition, such as rheumatoid arthritis or lupus
• genetic and environmental factors
• some types of cancer.

Vasculitis is rare and can affect people of any age, sex or ethnicity, though certain specific types are more common in particular age groups. It's most often diagnosed in adults over 50. Smoking and using recreational drugs, such as cocaine, can also increase your risk.

Are there different types of vasculitis?

Yes. There are more than 20 named types of vasculitis, usually grouped by the size of the blood vessels affected: large, medium or small. Some of the most common are described below.

Giant cell arteritis (also called temporal arteritis) is a large vessel type that inflames the arteries at the side of the head. It mostly affects adults over 50 and can cause headaches, jaw pain when chewing, and vision problems. It needs urgent treatment, as it can lead to permanent vision loss or stroke if untreated.

Takayasu arteritis is a rare large vessel type affecting the aorta and its main branches, mainly in young women. It can cause tiredness, weight loss, and painful, numb or cold limbs.

Polyarteritis nodosa is a medium vessel type that particularly affects the arteries supplying the gut, kidneys and nerves, usually in middle-aged people. It can cause abdominal pain, muscle and joint pain, and numbness or tingling.

Kawasaki disease is a medium vessel type that mainly affects children under 5, causing a high temperature lasting 5 days or more, along with a rash and swollen glands.

Granulomatosis with polyangiitis and microscopic polyangiitis are small vessel types that mainly affect middle-aged or older people. They can affect the nose, sinuses, ears, lungs and kidneys, causing symptoms such as nosebleeds, sinus problems, coughing up blood, and kidney problems.

IgA vasculitis (also called Henoch-Schönlein purpura) is a small vessel type that's more common in children. It typically causes a bruise-like rash, joint pain and tummy pain, and usually improves without treatment.

Behçet's disease affects arteries of different sizes and typically causes mouth and genital ulcers. It's more common in people from Greece, Turkey, the Middle East, China and Japan.

How is vasculitis diagnosed?

Vasculitis can be difficult to diagnose because its symptoms can look like those of many other conditions. Your doctor will start by asking about your symptoms and taking your medical history, then carry out a physical examination.

Tests that can help confirm the diagnosis and check which organs are affected include:

• blood and urine tests, to look for signs of inflammation and check how well your kidneys are working
• scans such as an ultrasound, CT or MRI, to look at your blood vessels and organs
• an angiogram, a special X-ray that uses dye to show narrowing or blockages in blood vessels
• a biopsy, where a small sample of affected tissue is removed and examined under a microscope.

A biopsy usually gives the clearest confirmation, but isn't always possible, and treatment shouldn't be delayed while waiting for one if your symptoms need urgent attention.

How is vasculitis treated?

Treatment aims to bring the inflammation under control and then keep it under control to prevent flare-ups. Which treatments you need depends on the type of vasculitis, how severe it is, and which organs are affected.

Non-surgical treatment options include:

• steroid medicine, usually the first treatment tried, to reduce inflammation
• other medicines that reduce the activity of the immune system, such as methotrexate, azathioprine or cyclophosphamide, for more serious cases
• newer biologic medicines, such as rituximab, which target specific parts of the immune system and are used for certain types of vasculitis
• for severe cases, plasma exchange (filtering your blood plasma) or immunoglobulin therapy given through a drip.

Surgical treatment is less commonly needed, but may be used to repair or bypass a damaged blood vessel, or to treat organ damage caused by vasculitis, such as a kidney transplant if the kidneys have been severely affected.

When should I see a doctor?

See your doctor if you have ongoing or worsening symptoms such as fatigue, fever, rashes, joint pain, or numbness and tingling, especially if you don't know what's causing them.

Seek urgent medical help (call 999 or go to A&E) if you have:

• sudden vision loss, double vision, or a new severe headache, particularly if you're over 50, as this can be a sign of giant cell arteritis, which can cause permanent blindness or a stroke if not treated quickly
• sudden difficulty breathing
• symptoms of a heart attack or stroke.