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Behcet syndrome

Behçet’s disease, (formerly known as Behçet’s syndrome), is a chronic condition due to disturbances in the body’s immune system. This system, which normally protects the body against infections by producing controlled inflammation, becomes overactive and produces unpredictable outbreaks of exaggerated inflammation. This extra inflammation affects blood vessels, usually the small ones. As a result, symptoms occur wherever there is a patch of inflammation, and can be anywhere where there is a blood supply. (Taken from

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History

Behçet's disease is named after Hulusi Behçet (1889-1948), the Turkish dermatologist and scientist who first recognized the syndrome in one of his patients in 1924 and reported his research on the disease in Journal of Skin and Veneral Diseases in 1936. The name (Morbus Behçet) was formally adopted at the International Congress of Dermatology in Geneva in September 1947.

Benedikt Adamandiades, a Greek ophthalmologist, reportedly described the disease six years before Behçet did. Nevertheless, only Greek scientists refer to the disease Adamantiades-Behçet's or even Adamantiades' disease.

Behçet's disease was probably first described by Hippocrates in the 5th century.

Pronunciation note

Because it contains a cedilla, "Behçet" is frequently wrongly assumed to be French in origin and pronounced with a sibilant "s" sound (as in "satsuma") or soft "ch" (as in "shoe"), with and the "t" incorrectly silenced: "Beshay". Because Hulusi Behçet was Turkish, the correct pronounciation is with a hard "ch", as in "church", and with the terminal "t" sounded: "Bet-chet".

Pathology

The symptoms of Behçet's disease are believed to be caused by an over-active immune system which, without any apparent infections, produces recurrent outbreaks of inflammation in small blood vessels. Common symptoms include mouth ulcers, sore genitals and eye inflammation, and arthritis in older patients, mostly painful but not life-threatening conditions. However, some patients may be unable to work because of the pain and the impaired vision and mobility. In some severe cases, uncontrolled inflammation may lead to blindness, intestinal complications, stroke, and even meningitis, which can be fatal.

This disease usually first strikes patients in their 20s and 30s. It then becomes a fluctuating lifelong disorder with a series of remissions and exacerbations which can be from days to months. Complete remission is rare.

Diagnosis

There is no specific pathological test for Behçet’s disease at present. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks. Other causes for these symptoms have to be ruled out before making the diagnosis. The symptoms do not have to occur together, but can have happened at any time.

There are three levels of certainty for diagnosis:

  1. International Study Group diagnostic guidelines (very strict for research purposes)
  2. Practical clinical diagnosis (generally agreed pattern but not so strict)
  3. 'Suspected' or 'Possible' diagnosis (incomplete pattern of symptoms)

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Cardiac Thrombus in Behcet Disease - Letter to the Editor
From CHEST, 8/1/01 by Satoru Yoshida

To the Editor:

We read with interest the review by Mogulkoc and colleagues (August 2000)[1] regarding intracardiac thrombus in 24 patients with Behcet disease previously published in 21 reports. It is also noted that the authors suggest a unique geographic distribution of this condition in the Mediterranean basin and the Middle East. However, several important articles[2-4] from the Far East are not mentioned in the review article, although it is well-known that Japan is one of specific areas to have a high prevalence of Behcet disease. We report herein a case of Behcet disease with cardiac and pulmonary arterial thrombus from a radiologic point of view.

A 19-year-old Japanese man was admitted to our hospital in January 1996 due to hemoptysis and right heart failure. He had undergone the surgical removal of right ventricular thrombus at another hospital 9 months previously. He received a diagnosis of incomplete Behcet disease based on oral ulceration, skin lesion, uveitis, and positive human leukocyte antigen-B51. Enhanced helical CT (Fig 1, top, middle) revealed thrombus of the right atrium, right ventricle, and right pulmonary artery. Indium-ill platelet scintigraphy (Fig 1, bottom) showed strong uptakes in the right-sided heart and right pulmonary hiatus suggesting acute thrombus formations. Scintigraphy clarified the activity of thrombus and contributed to determining the therapeutic strategy against the serious complications of Behcet disease.

[ILLUSTRATION OMITTED]

Helical CT can be helpful in the assessment of thoracic manifestations of Behcet disease including thrombus of the systemic veins, heart, and pulmonary arteries.[2] To our knowledge, there have been no reports of the use of indium-111-labeled platelet scintigraphy in cardiac thrombus in Behcet disease.[1-5] Scintigraphy can be used to determine whether a thrombus is acute or chronic.[6]

The selection of key words for a computerized search of the medical literature using the National Library of Medicine's MEDLINE is important. Our case and several reported cases[2-5] strongly suggest that the Far East should be included among the specific geographic distribution of patients with cardiac thrombus in Behcet disease.

Correspondence to: Satoru Yoshida, MD, Department of Radiology, Muroran City General Hospital, Yamate-chou 3-8-1, Muroran 051-8512, Japan; e-mail: satyoshi@chive.ocn.ne.jp

REFERENCES

[1] Mogulkoc N, Burgess MI, Bishop PW. Intracardiac thrombus in Behcet's disease: a systematic review. Chest 2000; 118: 479-487

[2] Ahn JM, Im JG, Ryoo JW, et al. Thoracic manifestations of Behcet syndrome: radiographic and CT findings in nine patients. Radiology 1995; 194:199 -203

[3] Imai H, Sakakibara M, Yoshida K, et al. Magnetic resonance imaging of cardiovascular thrombi [in Japanese]. J Cardiogr 1985; 15:681-694

[4] Yoshimura H, Ishii J, Watanabe N, et al. A case of cardiovascular Behcet's disease detected as multiple nodular shadows on chest X-ray [in Japanese]. Nihon Kyobu Shikkan Gakkai Zasshi 1997; 35:1074-1079

[5] Yasuo M, Nagano S, Yazaki Y, et al. Pulmonary embolism due to right ventricular thrombus in a case of Behcet's disease. Jpn Circ J 1999; 63:909-911

[6] Kight LC. Scintigraphic methods for detecting vascular thrombus. J Nucl Med 1993; 34:554-561

COPYRIGHT 2001 American College of Chest Physicians
COPYRIGHT 2001 Gale Group

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