"Behcet's disease is characterised with oral aphthosis, skin lesions, genital ulcers and ocular lesions. In addition, some patients may develop other various symptoms in almost all parts of the body. Involvement of the central nervous system, large (blood) vessels and intestinal tract can be life-threatening or leave functional disability.
Patients who have such serious manifestations are categorised into the Special Types (neurological, vascular and intestinal types) of the disease according to the Japanese criteria, and should be intensively treated with high dose of corticosteroids and/or immunosuppressants. Operative indication is considered in patients having massive intestinal bleeding, intestinal perforation, rapture of aneurysm and complete occlusion of large arteries and veins.
Unfortunately, it is impossible to anticipate the development of the special types of the disease. Patients who are suffering from suspicious symptoms should consult their attending physician. MRI of the brain, angiographic and colonoscopic examinations are helpful in detecting the lesions and establishing the therapeutic plan."
This slide illustrates the distribution of lesions caused by Behcet's Disease. In addition to common symptoms, such as uveitis or aphthosis, genital ulcers and skin lesions, various symptoms occur in any part of the whole body. This is another feature of Behcet's Disease.
This slide shows the frequency of the individual symptoms in patients with BD. We compared the data of the large scale regional study of Japan, Germany, Turkey and Greece. I'm sorry, we don't have the data from Jordan. These diagnostic symptoms, which are included in the International Criteria proposed by the International Study Group for BD- we cannot find the minor symptoms among them. So today my talk is focusing on the other things, or minor symptoms of this disease. Ulceritis and epididymitis, and Gastro Intestinal symptoms. Gastro lesions can occur in patients with BD. The frequency of these symptoms is around 10-20%. However, these three involvements can cause serious outcomes, and need intensive therapy.
I'll give you the definition of the special types of BD, as decided upon in Japan. Patients who are categorised into special types present serious symptoms, based on the intestinal tract, large vascular tract or Central Nervous System region. All of them can be present, or lead to irreversible serious symptoms. Therapeutic priority should be given to these symptoms.
There are three special types of Behcet Disease. First, Intestinal-BD, second is Vasculo-BD, and third is Neuro BD.
Rupture of aneurysm can be fatal. The intestinal lesions cause abdominal pain, diarrhea and melena. The ileocaecal region is the most commonly affected part.
This is a schematic figure of the digestive system, from the mouth to the anus. These are organs particularly affected by Behcet Disease. The lesions are distributed in almost all of the digestive system. However, here and here is the most frequently affected. This region is located in the right lower part of the abdomen. The patient presents abdominal pain, diarrhea and constipation, and sometimes bloody stools.
This is a graphic study of the colon. Here is the caecum and here is the ascending colon and this is the transverse colon. You can see the ring form of normal shadow here, indicating the presence of ulcerative lesion in the caecum.
These are endoscopic findings. Around here the mucousal membrane has a normal appearance. However, you can see an oval whitish mark here. This is an ulcer. This is a giant ulcer in the caecum. This ulcer is associated with bleeding. This yellowish part is ulcerative.
Vascular lesions occur in any size of arteries and veins. Occlusion of large arteries or veins cause circulatory insufficiency in the regional tissues.
This slide shows the frequency of large vascular lesions in patients with BD. As Dr. Madanat showed, BD may happen in both arterial and venous regions. And the lesion is sometimes an occluded lesion, and sometimes it can cause aneurysm. Venous lesions are usually occlusive.
This slide shows the dilation of a superficial abdominal vein. You can see the vein like a snail-dance. This is not the primary lesion in this patient. This patient has obstruction of large veins in the abdomen, and blood cannot return through the abdominal vein. Instead of the abdominal vein, the blood returns to the heart through the superficial abdominal vein, so his veins are dilated.
This slide also shows a dilated superficial vein. This is a very important sign, indicating the presence of obstruction of a deep vein.
Before showing the angiographic findings in patients, I'll give you a small lecture for the normal structure of the aorta. The aorta begins here. In fact, here is the heart, and the aorta starts here, and makes the aortic arch part and then descends, and the aorta ends in the pelvis, giving two branches, right and left commoilial artery. This is normal structure. And in the next two slides I'll show you the typical angiographic findings in patients with BD. One patient has a complete obstruction of the branch of the aortic arch, here, and the other patient has a complete obstruction of the left commoilial artery.
This slide shows the angiographic findings of the aortic arch. So you cannot find the third branch here. There must be three branches here. However, this is the first, this is the second, but you cannot find the third branch. The third branch is completely obstructed. This is the left suprarenal artery, supplying the left arm and part of the head. So this patient has a permanent tiredness of the left arm and dizziness when he moves his left hand.
This slide shows another example of complete obstruction of the major part of the aorta. The aorta ends here, and gives right and left commoilial artery. However, you cannot find any branch here. This finding indicates complete obstruction of the left commoilial artery at the origin.
This slide shows aneurysm of the leg artery. This mushroom form here indicates that aneurysm in Behcet comes from the anterior tibial artery.
This is a cardiography in a patient with BD, who suffered from heart failure. Here is the heart, the cavity of the heart, and this is the aorta. You can see the abnormal finding here. This is an aortic barrier between the heart cavity and the aorta. Patients with BD may have cardiac disease like this.
This is a pulmonary ventilation scintigram, showing the complete event of right upper lobe. The black dots that should be there are not seen. This finding indicates the complete obstruction of the pulmonary artery in the right upper field.
Neurological manifestations caused by cerebral and brain-stem lesions include acute meningitis and progressive motor paralysis. Psychiatric symptoms such as personality changes and dementia are refractory to any therapies in the terminal stage.
This figure illustrates the normal structure of the central nervous system, and the anatomical sites, which are most commonly affected in patients with BD. This is the meningium and this is the cerebrum, and here is the brainstem. These three anatomical sites are most commonly affected in patients with BD.
Now we are going to look at the different parts of the brain and the symptoms they present. We see that when the meningium is strongly involved the patient has meningitis. High fever and strong headache are typical. The patient may also lose consciousness.
The brain stem can present symptoms that may lead to death. Motor disturbance and sensory disturbance are also seen. Cranial nerves are distributed in the face, head and throat, and we can sometimes see cranial nerve palsy in patients with brain stem involvement.
When the patient has cerebral disease, the patient presents psychiatric disorders, including dementia and personality changes. This is the most refractory stage in CNS involvement.
We have several usual examinations for detecting neurological symptoms. Analysis of cerebral particulates is essential for making diagnosis of meningitis. We need to exclude meningitis caused by other etiology. MRI and CT are useful to detect lesions. MRI is the most powerful strategy for detecting lesions. Electroencephalography and Cerebral Blood Flow are also useful.
This here is typical MRI findings in patients with BD. In principle, the structure of the brain should be symmetrical. If you find asymmetrical structure, that must be abnormal. Here you can see a bright spot. This is a lesion of BD, which is located in the pons, a part of the brain-stem.
This is another view of the MRI findings in a patient with BD. Here is the front. This is, maybe, the nose, and this is the neck. you can find a white spotty lesion here. This is located in the brain stem.
This is a series of MRI examinations in a single patient. We conducted an MRI study on this patient. At that time, there was no abnormality, no abnormal findings. However, one year later, the patient developed sensory disturbances, and presented neurological symptoms. The MRI detected a whitish spot here. The patient was treated with corticosteroids. After successive treatment, the lesion disappeared. In some cases, the lesion is irreversible, left for a long time even after successive treatment.
This slide shows the principals of therapy of the special types of BD. Patients with intestinal BD are treated with corticosteroids and sulfusalazine. When the patient has vast spreading or intestinal perforation, surgical operation should be considered. For vascular BD, corticosteroids and immunosuppressants are employed. Anticoagural and antiplatelets, which reduce the coagurality in patients, is another important supplemental therapy. When the patient has a rupture of the aneurysm, of course the patient needs surgery. So when a patient has actual disability due to the occlusive lesion, the patient needs surgery again. And percutaneus transluminal angioplasty means catheter surgery. Neuro BD is treated with corticosteroids and immunosuppressants. Sometimes with antiplatelets. However, patients with progressive neurological disease are resistant to any therapy. In those cases symptomatic therapy is most important.
Still we have no satisfactory therapeutic strategy for the serious forms of BD. However, as Prof. Ohno mentioned previously, we are now developing new strategies, including antiTNF-alpha therapy. We would like to establish new strategies for patients suffering from serious symptoms. (Dr. Tsuyoshi Sakane and Dr. Mitsuhiro Takeno, May 2000).