| BEHCET'S ONTARIO Canada |
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| *Diagnostic Criteria and Symptoms |
| What are the "International Criteria for Behcet's Disease"? The International Criteria for Classification of Behcet's Disease were adopted in 1989, at the Fifth International Conference on Behcet's Disease. They are symptoms that are most likely to indicate a case of Behcet's, in the absence of any other medical explanations. The originators recommended that the symptoms list be known as "classifications" criteria rather than "diagnostic" criteria -- they felt that the list was more useful as a way to group patients for Behcet's research studies rather than as a way to diagnose individual cases of BD. However, because of a lack of laboratory tests specific for Behcet's, and with limited clinical experience in treating BD patients, many physicians lean heavily on the International Criteria to help with a diagnosis. Can you be diagnosed with Behcet's and not have the necessary symptoms listed? Yes! This can happen if a physician is very familiar with the diagnosis and treatment of Behcet's, and understands the relative importance of other serious, unlisted symptoms that the patient may be experiencing. One paper by Lueck et al (1993) described a patient with uvietis and widespread CNS involvement, who was diagnosed with neurosarcoidosis. After the patient's death, an autopsy discovered microscopic tissue changes that indicated a Behcet's diagnosis after all, even thought the patient had never suffered from oral or genital ulcerations or artheritic pain. So it is possible (but rare) for a patient to have Behcet's without the hallmark oral ulcers. Unfortunately, at the opposite end of the spectrum, some physicians who are unfamiliar with Bd have been known to base a Behcet's diagnosis on nothing more than a single bad case of mouth ulcers. Are there other lists of Behcet's symptoms beside the International Criteria? Yes, there are at least six other Behcet's criteria lists, orgination from various countries around the world. Each list tends to highlight BD symptoms that the originators feel are important in their specific geographic area. While the International Criteria are generally used to classify research subjects for Behcet's studies, individual doctors may choose any one of the criteria lists to help them diagnose their patient. According to Lee(2001), however, the International Criteria have one specific drawback -- the requirement that patients show recurrent oral ulcers before receiving a Behcet's diagnosis. In his book, Behcet's Disease: A Guide to its Clinical Understanding, Lee lists several studies where BD patients have shown no oral symptoms; this situation may delay proper diagnosis and treatment. He therefore suggests that two diagnostic criteria be used by physicians at the same time, to compensate for drawbacks inherent in any one specific set of criteria. Lee's recommendation is for use of the International Criteria, in combination with the revised (1987) criteria of the Behcet's Syndrome Research Committee of Japan; What types of symptoms show up most frequently in Behcet's patients? Kaklamani and Kaklamanis (2001) complied the results of six specific studies preformed on a total of 8,039 BD patients between 1970 and 2001. Symptoms Prevalence: Oral ulcers 96-100% of cases Genital ulcers 64-82% Skin lesions 74-87% Ocular lesions 47-73% Arthritis 40-64% CNS involvement 3-20% Vasculitis 6-38% GI Involvement 4-20% Epididymo-orchitis 6-22% Positive pathergy test 37-62% CNS=centeral nervous system; GI=gastrointestinal *One study of 601 patients (7% of total 8039 patients reviewed) did not reprot on GI involvement **Two studies totalling 1097 patients (14% of total 8039 patients reviewed) did not report on epididymo-orchitis ***One study of 496 patients (6% of total 8039 patients reviewed) did not report on pathergy test results Why do the statistics look like that? for example, why does the list say that "3-20%" of patients have CNS involvement? The statistics in this example show a range of the results from six different research studies that were preformed in different countries. In the case, the fewest number of patients with CNS symptoms in any of the reviewed studies was 3%. The greatest number of patients with CNS symptoms in any of the studies was 20%. So - if there were 100 patients in each of these studies, then three patients had CNS symptoms in the first study, and twenty paitents had CNS problems in the last study. [As an aside, Kocer etal (1999) made mention of a 4-49% range of CNS involvement, showing that results can vary widely between research studies. In addition, research results can be influenced by the location of the original study and the heritage of the enrolled patients, as some symptoms can be seen more commonly in some parts of the world than in others] *Copyright 2003 by Joanne Zeis, from "Essential Guide to Behcet's Disease", and is used with permission. |