WHAT IS BEHÇET’S DISEASE?
Where does the name come from? Behçet’s Disease was first defined by Prof. Dr. Hulusi Behçet in 1937 as a disease causing recurring aphthous ulcers in the mouth and genital organs as well as uveitis in the eye. However, Behçet’s Disease may involve almost all systems of the body, affecting joints, blood vessels, respiration, central nerve system and digestive system organs. It results from an abnormal reaction of the immune system against small vessels. The findings and symptoms of the disease vary depending on the organ and system involvement, and some symptoms last for a long time, yet there are also calm periods during which no symptoms are observed.
OCULAR INVOLVEMENT IN BEHÇET’S DISEASE AND ITS SYMPTOMS
Ocular involvement in Behçet’s Disease may be observed as anterior uveitis, posterior uveitis or “panuveitis” involving anterior and posterior uveitis combined. Patients with ocular involvement may have the uveitis symptoms described above, including “hypopyon” which is more frequent in Behçet’s disease patients and indicates the probability that the disease may progress in a way causing severe damage (see Photograph 1).
WHAT IS THE PREVALENCE OF OCULAR INVOLVEMENT IN BEHÇET’S DISEASE AND WHEN DOES IT OCCUR?
Ocular involvement is observed in about 45-55% of Behçet’s disease patients. These rates have been calculated on the basis of those patients who referred to health centers, thus are not indicative of the actual rate in the society. Behçet’s Disease may be diagnosed upon the onset of the ocular disorder. This may be due to the fact that other systemic findings may not have forced the patient to seek medical care. It may also be the case that patients diagnosed upon other systemic findings develop ocular involvement later, yet such rate is quite low. Ocular involvement may start in a single eye and continue there. However, the disease mostly involves both eyes.
WHO ARE UNDER HIGH RISK IN BEHÇET’S DISEASE IN TERMS OF OCULAR INVOLVEMENT AND SEVERITY?
Ocular involvement in Behçet’s Disease and severe uveitis which is the ocular occurrence of the disease, hence serious vision loss are associated with early onset of the disease and male gender. Ocular involvement is more frequent in men than women. Ocular involvement is also an indication of the fact that the disease will have severe progression. The most significant sign showing that the ocular disease will progress in a serious and damaging way is the frequent and intense progress of exacerbation symptoms called activation.
How does ocular involvement progress in Behçet’s Disease and which factors stimulate uveitis attacks? The severity and frequency of the attacks which are a characteristic of the clinical manifestation of ocular involvement, vary in each case. Appearance and disappearance of such attacks cause permanent damage in the normal tissues and structures of the eye that leads to permanent vision loss which is the most important prognostic evaluation of uveitis. There is no preliminary sign for the occurrence of uveitis attacks. It is very important to keep the disease in close follow-up, arrange therapies according to the clinical changes observed in follow-ups, conduct therapies under a protocol and switch to binary therapy in case the parameters of the protocol cannot be satisfied. Stress, fatigue, irregular use of medication and inflammatory diseases may stimulate uveitis attacks. Particularly, failure to regularly use the medications in the way recommended may cause exacerbation of the disease. Accordingly, the patients should be constantly warned about the importance of regular participation in treatment.
IS TREATMENT REQUIRED IN EVERY PATIENT WITH OCULAR INVOLVEMENT IN BEHÇET’S DISEASE?
The inflammation of the vitreous which fills the vitreous cavity at the back of the ocular lens is known as vitritis. Patients diagnosed with only vitritis as an ocular symptom may be left without treatment. In patients with anterior uveitis, topical eye drops and pomades with corticosteroids may be applied in order to suppress inflammation. Pupil-dilating eye drops may be administered at prescribed intervals to separate the adhesion between the iris and the ocular lens. Patients carrying the risk of developing adhesion may be given medication that strongly dilate the pupil at more frequent intervals. As for patients with posterior uveitis or panuveitis, systemic corticosteroids and immunosuppressive drugs may be used in certain doses singly or in combination depending on the severity of the disease. Systemic corticosteroids and immunosuppressive drugs are also used in other severe forms of the disease, such as major vessel and neurological involvement.
WHAT IS THE RISK OF VISION LOSS IN BEHÇET’S DISEASE?
During attacks, reduced vision may be experienced due to the temporary damage in the ocular tissues. Following repetitive and severe attacks, permanent vision loss may develop because of the damages that occur especially at the back layers of the eye. Long-term maintenance of a good level of vision is possible in patients experiencing ocular involvement in a way not damaging the visual cortex and/or in female patients. Higher risk of vision loss in countries such as Turkey and Japan results from the higher number of patients with Behçet’s disease. In other countries such as North America, Behçet’s disease is less common.
HOW SHOULD PATIENTS WITH OCULAR INVOLVEMENT IN BEHÇET’S DISEASE BE FOLLOWED?
Patients with ocular involvement are kept in regular follow-up by uveal disease departments of ophthalmology clinics. These patients are urged to be careful about the emergence of new symptoms and asked to immediately refer to their respective ophthalmology clinics upon any suspicion of new attacks. Patients’ regular participation in follow-up is significant for following the progress of the disease, determining the form, dose and side effects of drug therapy and detecting the complications of the disease.
