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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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Some Historical Notes on Behcet's Disease
From CHEST, 2/1/01 by Tsung O. Cheng

To the Editor:

I read with interest the review article (August 2000) on intracardiac thrombus in Behcet's disease (BD) by Mogulkoc et al.[1] Having recently returned from a visit to Turkey and Greece, I would like to make some comments, especially from the historical point of view.

First, Hulusi Behcet was a Turkish dermatologist from Istanbul (1889-1948). To commemorate his contribution to the syndrome that now bears his name, the University of Istanbul, where I served as a Visiting Professor during my recent visit, named the medical library after him (Fig 1).

[Figure 1 ILLUSTRATION OMITTED]

Second, having visited the island of Kos, the birthplace of Hippocrates, during my visit to Greece, where I served as a Visiting Professor at the University of Athens, I came to recognize that the first description of the symptomatology of BD was actually reported by Hippocrates.[2] In fifth century BC, Hippocrates wrote in his third book of epidemiology[3]:

Third, although Mogulkoc et al[1] did not mention mitral valve prolapse (MVP) in their article, there is a high incidence of MVP in patients with BD, occurring in 50% of cases.[4,5] The association of MVP and BD was first reported in China.[5,6] Shen et al,[5] from Shanghai, China, postulated that MVP may have been the result of structural damage and functional derangement caused by the underlying vasculitis of BD. Whereas the association of MVP and BD may be either causally related or coincidental, it is important to keep in mind that, although BD is relatively rare in the United States, MVP is very common.

BD occurs most frequently in Japan and in the Mediterranean countries, and also in the population linking these two areas to each other. It occurs most frequently between latitudes 30 [degrees] and 45 [degrees] north, in Asian and Eurasian populations. This area coincides with the old Silk Route. Thus, BD is often also called "Silk Route disease."[6]

Correspondence to: Tsung O. Cheng, MD, FCCP, Professor of Medicine, The George Washington University, 2150 Pennsylvania Ave NW, Washington, DC 20037-3201

REFERENCES

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

[2] Cheng TO. Behcet's disease [letter]. N Engl J Med 2000; 342:588

[3] Kaklamani VG, Vaiopoulos G, Kaklamanis PG. Behcet's disease. Semin Arthritis Rheum 1998; 27:197-217

[4] Morelli S, Perrone C, Ferrante L, et al. Cardiac involvement in Behcet's disease. Cardiology 1997; 88:513-517

[5] Shen L-L, Cui G-G, Liang R-L. Valve prolapse in Behcet's disease. Br Heart J 1985; 54:100-101

[6] Cheng TO. Behcet's disease ("Silk Route disease") and mitral valve prolapse [letter]. Cardiology 1998; 89:306

COPYRIGHT 2001 American College of Chest Physicians
COPYRIGHT 2001 Gale Group

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