Behçet's Disease or Behçet's Syndrome:
-Considerations for the Unified Diagnosis Related Terminology-By Sungnack LEE (Korea)
The clinical manifestations of what is known today as Behçet's disease were described in as far back as the era of Hippocrates. However, in 1937, the Turkish dermatologist Hulusi Behçet was the first to give a comprehensive description of the symptom-complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis as a disease entity, which today bears his name.
During the last six decades, more than 4,500 references related to Behçet's disease were published (1998).1 It reflected results of the strong research activities and the worldwide interest in this unique disease. However, we Behçetologists have not yet found a consensus for a unified terminology. In other words, we have used different medical terms to describe this condition such as Behçet's disease, Behçet's syndrome, or others for nearly sixty years.
In fact, the difference occurred mainly due to a single matter: whether to use the term "Syndrome" or "Disease".
To reach an agreed terminology, the author's group reviewed a list of published papers from the past sixty years that had used medical terms like syndrome or disease for Behçet's condition.
The Ophthalmotogist T. Jensen (1941)2 had confirmed Behçet's observations through his clinical case report, and he became the first author to mention the terminology
According to our studies, among 2,228 publications related to Behçet's condition over the past sixty years, 1,591 papers used the term "disease" in the title (71.40%) while 637 used the term "syndrome" (28.59%).
Furthermore this was a time related phenomenon. Among the 112 publications between 1941 and 1969 the term "disease" was used in slightly less than half (47%) of the cases. However this percentage rose up to 82% among 986 articles published between 1990 to 1998.
Recently we sent out survey questionnaires to 34 board members of the International Study Group for Behçet's Disease. Although the total number of respondents was relatively limited, the professional qualities of our respondents were highly sophisticated so that the significance of the responses could be highly valued.
Of the 34 questionnaires, which were sent, 22 responses were collected:
The first question was: Should we have an agreed terminology regarding the Behçet's-condition? Yes: 21/23 responders (91.30 %); No: 2/23 responders (8.69%)
The second question was: Which terminology is commonly applied in your country? Behçet's syndrome: 3/23 (13.04 %); Behçet's disease: 15/23* (65.21%); Both: 4/23 (17.39%)
The third question was: Which terminology do you prefer personally? Behçet's syndrome: 8/23 (34.78%); Behçet's disease: 14/23* (60.86%); Both: 1/23 (4.34 %)
* One responder used the term Admantiades-Behçet's disease. Since our survey's purpose was to know whether the term disease or syndrome should be applied to the Behçet's condition, the mentioned case was included with the term "disease"
The respondents presented some remarkable comments.
In conclusion our study group firstly suggests that it is time to come to an agreed consensus for a unified terminology.
Our study group further suggests that, based on the findings of the different studies, all of us, as Behçetologists should declare to use only one diagnostic terminology: Behçet's disease.
Our general image as Behçetologists without a defined diagnosis related terminology could not be viewed favorably by the whole medical world. We should seriously concern the fact that we do not even have an agreed diagnostic terminology yet.
Treatment of Behçet's SyndromeBy Sebahattin YURDAKUL &
Vedat HAMURYUDAN (Turkey)
Current treatment of Behçet's syndrome (BS) still continues to be symptomatic and empirical but most would agree that the outcome of our patients is better today than it was 20-30 years ago. Although the available agents used in the treatment of BS remain almost (with the exception of alpha-interferon) unchanged during the last two decades, we use them with more skill these days and most of that skill comes from information obtained in clinical trials.
There is still considerable difference even among the experts in their approach to the treatment. A survey, which was conducted in the last International Congress in Reggio Emilia, has demonstrated the lack of consensus on the controversial issues of treatment.1 Another example to this marked divergence is reflected in a recent review on BS.2 In this review, colchicine has been recommended for preventing both anterior and posterior uveitis with a high degree of efficacy whereas the authors of this letter would hardly prescribe colchicine to their patients for the treatment of uveitis. We believe that these differences will continue until we know more about the pathogenesis, until more properly conducted drug trials are available and until we define reliable instruments for assessing disease activity and damage.
The treatment in Behçet's syndrome is tailored to the site and severity of disease manifestations. Mild orogenital ulcers are treated with reassurance and local application of steroids and anaesthetics. On the other hand we know that male patients and those with a young age of onset (<25 yr.) generally have more severe disease course and require more aggressive treatment.
It is worth underlining that of the many currently used agents for the treatment various manifestations of BS only some (namely azathioprine, cyclosporine, thalidomide, colchicine and IF-a) have been shown to be beneficial in controlled clinical trials.
Of these drugs thalidomide has a prompt and powerful effect on oral and genital ulcers along with follicular lesions of BS. Additionally, it is the only drug that can induce complete relief of oral and genital ulcers.3 However, it does not seem to be a disease-modifying drug and relapses are seen after stopping treatment. Because of its severe side effects like teratogenecity and polyneuropathy, this drug should not be used routinely in the clinic. Until safe analogues of this drug are available, thalidomide should be reserved for severe cases resistant to other alternatives.
Interferon alpha, alone or in combination with low dose steroids, might be a powerful alternative for severe eye involvement of BS.4 Various studies have also reported its efficacy in the treatment of mucocutaneous and joint manifestations of BS. The effect of interferon starts relatively early but relapses usually come after stopping treatment.
It is worth mentioning here that an infectious etiology has been claimed for many years and various strains of streptococci have been in the forefront. A trial of penicillin from Ankara has already indicated promising outcomes especially in mucocutaneous disease and arthritis5 but clearly more work needs to be done.
An additional issue to be addressed in treatment is that of combination therapy. Although many centers use several drugs in combination (like colchicine with an immunosuppressive or azathioprine in combination with cyclosporine), there is precious little formal data on such treatment. This sort of formal experience is also definitely needed for better patient management.
In summary, recent years have seen substantial progress in the treatment of eye involvement and mucocutaneous manifestations of BS. Unfortunately we are still far from satisfactory in the treatment of life threatening manifestations of BS such as central nervous system and vascular involvement. Multi-center studies and improved methods in assessing patients will help to improve treatment of this mysterious disease. We hope that our newly formed International Behçet Society will be instrumental in the organization of such multicenter studies.