Behcet's disease and vasculitis
Vasculitis means inflammation of the blood vessels. Behcet's disease is one form of it and is more common in the Middle East. This page covers the signs of Behcet's, giant cell arteritis, and the times when you should not wait.
What is vasculitis?
Vasculitis means inflammation of the blood vessels. An inflamed vessel may narrow and carry less blood to an organ, so the signs depend on which vessels are involved. There are many types. This page covers two.
Behcet’s disease
Behcet’s is a rare disease with inflammation of the blood vessels and tissues. The exact cause is unknown, but genes and environmental factors play a part. It is more common in the Middle East, the Far East and Mediterranean countries, and symptoms often begin in a person’s 20s and 30s.
Signs
- Mouth and genital ulcers
- Red, painful eyes and blurred vision
- Acne-like spots
- Headaches
- Painful, stiff and swollen joints
The disease comes in waves: symptoms become severe and then settle. In severe cases there is a risk of vision loss or stroke.
Diagnosis
There is no definitive test. Blood and urine tests, imaging, a skin biopsy and a pathergy test are used to check for signs or rule out other causes. In a pathergy test the skin is pricked with a needle to see whether a particular red spot appears a day or two later.
As a rule, the diagnosis can be made with confidence if you have had at least three episodes of mouth ulcers in the past 12 months and at least two of these: genital ulcers, eye inflammation, skin lesions or a positive pathergy test. Other causes must also be ruled out.
Treatment
Behcet’s has no definite cure, but symptoms can often be controlled with medicines that reduce inflammation: steroids, immunosuppressants and biological therapies. The plan depends on your symptoms, and if the eyes are involved an eye specialist joins the treatment.
Giant cell arteritis (temporal arteritis)
In this disease the arteries, especially those at the side of the head, become inflamed. It is serious and needs urgent treatment, because it can lead to blindness and stroke.
Signs
- Frequent, severe headaches
- Pain or tenderness at the temples and scalp
- Jaw pain when eating or talking
- Vision problems, such as double vision or loss of vision
- Flu-like symptoms, weight loss and tiredness
Some of these patients also have pain and stiffness in the shoulders, neck and hips, called polymyalgia rheumatica.
Treatment
Treatment is with steroids and starts before the diagnosis is confirmed. A high dose comes first, then a lower dose for a long time, with regular follow-up. Never stop steroids suddenly.
When should you get help urgently?
- A new headache with temple or jaw pain, especially with changes in vision: be seen the same day.
- Eye pain, very red eyes, sensitivity to light or blurred vision: get checked the same day.
- Loss of vision: this is an emergency.
- Sudden weakness on one side of the body, a drooping face or trouble speaking: call 115.
See the full list of warning signs.
Frequently asked questions
Does every mouth ulcer mean Behcet's?
No. Mouth ulcers are very common and have many causes. Behcet's comes up when ulcers keep returning along with other signs, such as genital ulcers, eye inflammation or skin lesions.
Is there a test that confirms Behcet's?
No. There is no definitive test. The diagnosis comes from putting the signs together and ruling out other causes.
Why are steroids started for giant cell arteritis before the diagnosis is confirmed?
Because if this disease is not treated quickly, there is a risk of losing vision.
The first step: putting the signs together.
Go to the booking page or talk to support. Have your earlier test results and your medicine list ready.