Vasculitis

Vasculitis literally means 'inflammation of the blood vessels'.

Inflammation is your immune system's response to tissue that has become injured or infected.

Damaged cells release chemicals that cause blood vessels to leak fluid into the tissues, causing tissue swelling. If there's an infection, this helps isolate the invading germ.

In vasculitis, your immune system attacks the blood vessels by mistake. The trigger may be an infection, a medicine or another medical condition. Sometimes, however, the cause isn't clear.

It leads to swollen blood vessel walls and narrowed blood vessels. Blood flow to tissues and organs is reduced or blocked. If the blood vessel wall becomes weak, bleeding can occur.

Any blood vessel can become inflamed like this, from large arteries ("arteritis") to small blood vessels, in any place in the body. The larger the blood vessels affected, the more damage there may be.

All these variables mean there are many possible types of vasculitis, each with different symptoms and potential complications. These are covered below (ordered by size of the affected blood vessels):

These types of vasculitis are briefly explained below, with links taking you to more detailed information.

Giant cell arteritis

Giant cell arteritis is an age-related condition in which medium and large arteries, usually in the head and neck, become inflamed.

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

Symptoms of giant cell arteritis include:

aching and soreness around the temples
jaw muscle pain while eating
vision loss

It tends to only affect adults over the age of 50, and the cause is unclear. The main treatment is steroid medication (usually prednisolone), which is given promptly when the diagnosis is suspected.

Polymyalgia rheumatica

Polymyalgia rheumatica is another age-related condition closely related to giant cell arteritis. It causes pain, stiffness and inflammation in the muscles around the shoulders, neck and hips.

It may also cause more general symptoms, such as a fever, extreme tiredness, loss of appetite, weight loss and depression.

The condition is two to three times more common in women than men, and most people diagnosed with it are over 70 years old. The cause is unclear.

The main treatment is usually prednisolone (a steroid medication), which is usually used in lower doses than for giant cell arteritis.

Takayasu arteritis

Takayasu arteritis is rare in the UK. The main artery from the heart is affected, along with the major arteries branching off this.

These arteries gradually narrow over time, which reduces the blood supply to the limbs and other parts of the body. Treatment is usually steroid medication.

Read information about Takayasu arteritis from Vasculitis UK.

Polyarteritis nodosa

Polyarteritis nodosa is a rare form of vasculitis, in which the medium or smaller arteries become damaged and inflamed. It particularly affects the arteries supplying the gut, kidneys and nerves.

Sometimes, only part of the artery wall is affected, which becomes weak and may bulge, forming an aneurysm. If this leaks or ruptures, it can cause serious internal bleeding.

Polyarteritis nodosa can also affect the whole wall of the artery at a particular point, and cause a blockage.

The condition is potentially very serious. Typical symptoms are feeling unwell with fever, skin rashes, tummy aches, nerve damage and weight loss.

Polyarteritis nodosa can sometimes be triggered by an infection, but the exact cause is uncertain.

Read information about polyarteritis nodosa from the Great Ormond Street Hospital website.

Kawasaki disease

Kawasaki disease is a rare condition that affects small and medium arteries in children under the age of five.

The characteristic symptoms of Kawasaki disease are a high temperature that lasts for more than five days, with a rash, swollen glands in the neck, red fingers or toes, red eyes and redness of the lips, tongue or mouth.

Kawasaki disease can affect the coronary arteries (the blood vessels that supply blood to the heart), so some children with Kawasaki disease experience heart complications.

The trigger is thought to be an infection, although the exact cause is not fully understood. The affected child will usually need to be managed in hospital, because of the risk of complications – they will need to be carefully assessed for heart problems and treated with immunoglobulins and aspirin.

Granulomatosis with polyangiitis (Wegener’s granulomatosis)

Granulomatosis with polyangiitis is an uncommon disorder that mainly affects the blood vessels in the nose, sinuses, ears, lungs and kidneys.

It can cause a wide range of symptoms, including fever, night sweats, sinusitis, nosebleeds and crusting of the nose, shortness of breath, coughing up blood and kidney impairment.

Granulomatosis with polyangiitis is a serious condition that can be fatal if left untreated, because it can lead to tissue damage and organ failure.

High doses of drugs are needed to dampen down the immune system and bring the inflammation under control. Typically, it is treated with steroid medications (prednisone) and cyclophosphamide – a cancer drug that suppresses the immune system.

Read more about granulomatosis with polyangiitis .

Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome)

Eosinophilic granulomatosis with polyangiitis is a type of vasculitis that typically causes allergic sinusitis and asthma to develop in adults. It mainly affects adults aged 30-45.

Many of the other symptoms are general and include fever, muscle and joint aches, tiredness, loss of appetite and weight loss.

The condition can also affect the nerves, causing weakness, pins and needles or numbness, and it sometimes damages the heart muscle.

Read information about Eosinophilic granulomatosis from Vasculitis UK.

Microscopic polyangiitis

Microscopic polyangiitis is another rare and potentially serious long-term vasculitis.

Inflammation of the blood vessel walls may affect any organ, but particularly the lungs, kidneys and nervous system.

It can lead to raised blood pressure, kidney impairment, anaemia, breathlessness and coughing up blood.

Read information from the Oxford University Hospitals NHS website about microscopic polyangiitis.

Henoch–Schönlein purpura (HSP)

Henoch-Schönlein purpura (HSP) is a rare condition, usually seen in children, which results in a skin rash and joint and tummy pain. It can follow a recent infection.

Inflammation of the blood vessels can cause the small surface blood vessels to bleed into the skin, which results in a characteristic rash (purpura). Joint pain is also common.

HSP can also affect blood vessels in the bowel and the kidneys, and can cause tummy pain and visible blood in the urine or stools.

HSP usually gets better without treatment and is not usually serious.

Read more about Henoch-Schönlein purpura.

Less common types of vasculitis

Behçet's disease – typically results in mouth ulcers and genital ulcers, and is more common in people from Greece, Turkey, the Middle East, China and Japan
Buerger's disease (links to the Vasculitis UK website) – affects vessels in the legs and arms, leading to reduced blood flow to the hands and feet
Primary angiitis of the central nervous system (links to the Vasculitis UK website) – inflammation of the brain's blood vessels
Cogan's Syndrome (links to the Vasculitis UK website) – inflammation of the inner ears and eyes
Cryoglobulin-associated vasculitis (links to the Vasculitis UK website) – associated with cryoglobulins (proteins in the blood that stick together in the cold); can cause a rash on the lower limbs, joint pain or arthritis, nerve damage, abdominal pain and kidney failure
Hypersensitivity vasculitis (links to Vasculitis UK website) – most commonly caused by sensitivity to a drug such as penicillin, non-steroidal anti-inflammatory drugs or the contraceptive pill, and results in a skin rash
Vasculitis caused by rheumatoid arthritis (links to the National Rheumatoid Arthritis Society)

The links take you to more information on these rarer types of vasculitis.