Bright Sight

Oliver Backhouse, Consultant Eye Surgeon www.cataract.org.uk

Behcets Disease

Behçets disease (BD) is a form of inflammation which can affect many different parts of the body and whose cause is not fully known but a genetic predisposition is understood. It is rare in the UK but more common in the Middle East and Japan along the ancient trading Silk route. The name comes from a Turkish skin doctor, Hulusi Behçet, early last centuary. It usually causes inflammation inside the eye (uveitis) and patients are therefore often referred to the Uveitis clinic.

Diagnosing Behçets disease:

BD is a complex disease that can show itself in different ways in different patients. Sometimes it is very difficult to diagnose and there is no ‘test’ to confirm the diagnosis. For this reason there are some Internationally recognised signs and symptoms which together can make the diagnosis of BD. Not all of these problems need to occur at the same point in time; on the contrary, the diagnosis may only become clear after several years as different symptoms and signs show themselves.

1. Ulceration of the mouth.

Most of us have had a few mouth ulcers in our life. In BD, the ulcers can be large, frequent and occur in ‘crops’ of several at a time. They may be in unusual places such as lips, palate and throat. This is often the first sign.

2. Ulceration around the genitals.

These may be single or multiple. In women they can be external on the vulva or internally within the vagina. In men they can occur on the penis or scrotum. Sometimes soreness is noted but no ulcer is seen by the patient. Small scars may be left. If in doubt inform your doctor.

3. Uveitis.

This is present in over 70% of patients with BD. There are many cause of uveitis but the uveitis in BD has certain characteristics so it is not uncommon for the Ophthalmologist to be the person to make the diagnosis. The uveitis can be aggressive and needs vigorous treatment.

4. Skin Problems.

The skin is involved in 70% of patients with BD and can take on differing forms. The most common is an acne-like rash which may be in an unusual place (such as down an arm) and may form pustules. Another type of skin involvement is the presence of large (up to 3cm), tender, deep red / purple, slightly raised lumps called Erythema Nodosum. They are usually on the front of the lower legs but can be anywhere such as the face, arms or buttocks. Another type of skin involvement is when the skin surface becomes bruised or ulcerated. This is caused by inflammation of blood vessels (vasculitis) in the skin. A pustule may form at the site of skin trauma (Pathergy test)

These 4 types of inflammation above are called the 'major' manifestation of BD. If a patient has, or has had, all four types then the condition is known as ‘complete BD’. If a patient has only 2 or 3, then it is 'suspected BD'.

BD can also cause other problems but these are not needed in order to make the diagnosis. Other parts of the body are affected as BD is a general disorder of inflamed blood vessels. Many patients do not develop these other problems but if they occur they are important to recognize so appropriate treatment can be given. Approximately 50% will have gut involvement (pain, diarrhoea, constipation, ulceration), 40% can have transient, non-destructive joint pain which affects large joints more usually – Knee > ankle, elbow, wrist. Other problems are less common. Vasculitis can cause blockage of any blood vessel. In the lungs inflammation may cause chest pain; in the testes it may cause swelling and discomfort; in the head it can produce a headache or other problems such as a stroke; in the ear it can cause deafness. If in doubt always mention symptoms to your doctor.

UVEITIS IN BEHCETS DISEASE.

The uveitis in BD can be very characteristic. It may affect the front of the eye, the back of the eye or both together. If the front of the eye becomes inflamed there is often redness and pain and the eye may be sensitive to light. The vision may be blurred. If the back of the eye is affected there is no pain but, because blood vessels may become blocked, there can be marked reduction in vision. Both types of attack can arise rapidly, within a day or two. Either one or both eyes may be affected during an attack.

TREATING BEHCETS DISEASE.

It may be necessary to see more than one type of doctor as BD affects more than one part of the body. In the Uveitis clinic it will be possible to give general advice about the disease and the effects elsewhere in the body but, if necessary, a referral will be made to other doctors who are more expert in dealing with problems in their own field. At any one time it is important that one of these doctors is ‘controlling’ the treatment. As the uveitis can be severe, the Ophthalmologist is often in this position to ‘control’ treatment.

BD characteristically occurs in ‘attacks’ which can affect one or more areas. Sometimes these are mild and occur infrequently. In some patients with ‘severe BD’ the ‘attacks’ may be frequent or blend into each other so that here are some problems most of the time.

Treating attacks of uveitis:

During an attack of BD uveitis, steroids are needed to try and gain control of the inflammation. This may be in the form of drops with tables, local injection or by a vein in hospital. An information leaflet on Steroids (Prednisolone) is available in the clinic for you. Usually the severity of the attack can be reduced but permanent damage may have resulted especially following severe or cumulative attacks.

Preventing attacks of Uveitis:

Large amounts of steroids over long periods have their own problems. Because attacks can lead to cumulative damage, it is desirable to try and prevent attacks from occurring if possible. Other drugs such as Azathioprine and Cyclosporin are better at doing this. An information leaflet on Azathioprine and Cyclosporin is available in the clinic for you. Sometimes other newer drugs are needed. These drugs not only reduce the number off attacks of uveitis, they can be successful in suppressing most, or even all problems associated with BD.

The internet has further information and there are patient support groups. These groups can sometimes provide more detailed information. One such group can be contacted at: The Behçets Syndrome Society www.behcets.org.uk

Every patient and every eye inflammation is different. The information above is indicated to give you a general picture about uveitis in Behçets disease. Always ask the doctor or nurse in the clinic if you are unsure of, or want more detail on, any points in relation to your own eye problem and general health.

Disclaimer: Adapted from Manchester uveitis patient leaflet.