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European Journal of Echocardiography 2007 8(3):167-173; doi:10.1016/j.euje.2006.12.007
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Copyright © 2007, The European Society of Cardiology

The migration pattern, patient selection with diagnostic methodological flaw and confusing naming dilemma in Behçet disease

Cem Evereklioglu*

Department of Ophthalmology, Division of Uvea-Behçet Unit, Erciyes University Medical Faculty, Kayseri, Turkey

Received 1 December 2006; accepted after revision 15 December 2006.

* Tel.: +90 352 233 1565. evereklioglu{at}hotmail.com

Dear Editor, I have read with great interest the very recent review article of Leibowitz et al. (European Journal of Echocardiography 2006) titled "Echocardiographic manifestations of Adamantiades–Behçet's disease" and congratulate the authors for their work. They specifically evaluated cardiovascular involvement in Behçet disease that is characterized by myocardial disease, venous disease and disease of the aorta and great vessels. The authors reviewed the spectrum of echocardiographic imaging findings in Behçet disease patients and then suggested the use of echocardiography in such patients to comprehensively examine the great vessels pathology as well as myocardial and valvular performance. However, I would like to make some contributions to this review article and have a few vital questions to be answered by the authors of the present study.

First; The authors state both in the abstract and the Introduction section of the article that "the prevalence of Behçet disease is increasing in Western countries due to migration patterns". I think this statement should be proved and, therefore, be referenced with evidence based articles. Although this ubiquitous disorder exhibits a distinct geographic variation and is endemically higher particularly in Turkey, Asians and Japan,1,2 such a migration pattern stated by the authors has never been demonstrated for this disorder up to now. Indeed, Behçet disease accounts for up to one-fifth of cases of endogenous uveitis in some of these abovementioned countries that is most probably due to the association of environmental factors together with the histocompatibility antigen. Important in this respect is that Turkish individuals who have migrated to Germany demonstrate a lower risk of disease than those of the Turkish-origin population living in Turkey.2 Furthermore, the disease is virtually unknown in Japanese people who have emigrated to tropical Hawaii or the continental USA despite it having high prevalence in Japan.2 Therefore, I do not agree with the authors on this subject and it would be appreciated if they bring their evidence to the scientific community regarding their statement on "migration pattern".

Second; The authors state in the second sentence of the "arterial disease" section on page 2 that "the patient was initially diagnosed with Behçet disease at the age of 12 when he presented with carditis, arthritis, fever and oral and genital ulcers and was treated with steroids and colchicine". I disagree with the authors in this regard as the exact diagnosis of Behçet disease in a case depends primarily on the recognition of a characteristic set of sufficient symptoms and/or signs to allow the physician to diagnose the disease as Behçet disease based on the clinical sign constellation according to the "diagnostic criteria of International Study Group".3 This set of criteria, which was published in The Lancet in 1990, requires recurrent oral ulcers plus two of the four major symptoms or findings that follow such as recurrent genital ulcers, eye lesions, skin lesions and/or positive pathergy test. This classification omits the less certain features of the disease such as large vessel inflammation and arthritis. I mean that, in the absence of pathognomonic laboratory findings, the correct diagnosis of Behçet disease can be established only on the basis of the aforementioned strict rules that need the complete fulfillment of the criteria provided, and for which other etiologies for these manifestations have been strictly ruled out, including inflammatory bowel disease.4 Therefore, carditis, arthritis, fever and oral and genital ulcers of this 12-year-old patient do not fulfill the diagnostic criteria of this worldwide accepted classification system. Similarly, such a diagnostic insufficiency is also valid for the second 45-year-old male patient who was stated to have a history of oral aphthae and erythema nodosum in the article on page 3. Two major criteria for this man are clearly not sufficient for the diagnosis of Behçet disease according to International Study Group Criteria.3 Likewise, such a diagnostic flaw is, unfortunately, valid for the 20-year-old male patient with a history of scrotal ulcers and bilateral lower lobe pneumonectomy for pulmonary artery aneurysms stated on page 3, if there are no other symptoms characteristic for Behçet disease for this patient. Therefore, the authors should clarify such diagnostic confusions for the three patients in their review article.

Third; The authors indicate in the first paragraph of the Introduction section that Behçet disease was initially described by dermatologist Hulusi Behçet and ophthalmologist Benediktos Adamantiades, and further concentrated on the "classical clinical triad" in the following sentences. This statement is partly incorrect and I completely disagree with the authors that the disease was described initially by Benediktos Adamantiades. In fact, Dr. Adamantiades has never called the disorder "classical triad" or, strictly speaking, "triple symptom complex". We, as investigators, are primarily responsible during the selection and creation of our disease eponyms and titles that they should be consistent with our previous published international papers. I have carefully read the earlier papers of the present respected authors and all of their articles in the last three decades have called the disorder "Behçet disease" without exception,5–11 and none of the series of their papers has called the syndrome "Adamantiades–Behçet disease". Moreover, the European Journal of Echocardiography has published two other papers in 2002 and 2006 on the same subject, both of which used again the eponym Behçet,12,13 not Adamantiades–Behçet. Therefore, as an author interested in etiological, clinical and therapeutic research on Behçet disease,1,2,4,14–22 I was so disappointed to see that the disease was named for the first time as "Adamantiades–Behçet" by the authors when compared with their numerous previous articles. I would very much appreciate if Leibowitz et al. answer the most important query—why they are trying to change the name of a disorder well-known as "Behçet disease" or "Behçet syndrome" for more than 65years by every investigator, including the authors of the present article, interested in the care of this unique disorder in every country? I would also appreciate the authors bringing their historical evidence to the scientific community that must clearly and strongly be proved by clear references.

It is also clear that there should be a consensus between the articles of the same authors and journals over the years. Science does not accept personal feelings or personal usage and every name or disease has its special history with referenced proofs by positive science. I think the authors of the present paper might be writing in a biased manner while lacking sufficient knowledge about the whole historical process of the origin of "Behçet disease" in that it needs extensive revision and, therefore, important scientific corrections. Nonetheless, if Dr. Leibowitz and co-authors still believe or insist on the contribution of Dr. Benedictos Adamantiades during the discovery of this unique blinding disease, I think they should first know the whole historical evolution of manifestations and the naming process to such a degree as to make such a conclusion, as journals and book resources do not accept such a naming confusion. There have already been various naming efforts in the past for this disorder, such as "Gilbert's ophthalmia lenta",23 "Gilbert–Behçet disease",24,25 "Gilbert–Adamantiadis–Behçet disease"26 and Hippocrates–Adamantiades–Behçet disease,27 all of which were not accepted by the scientific community for the same reasons that will be discussed below in detail.

Dr. Adamantiades reported a case with relapsing hypopyon iritis, mucocutaneous lesions and arthritis concentrated in one male patient, and focused on the "recurrent ocular lesions" as we understand from its title where he defines his single patient as "recurrent hypopyon iritis"28 or "Sur un cas d'iritis à hypopion récidivant"29 that was the basis of the suggestion of "Adamantiades–Behçet" by a few authors today. However, Dr. Adamantiades was not the first to report such "recurrent eye lesions", "mucocutaneous symptoms" and "arthritis", and a number of male and female patients with "relapsing hypopyon iritis or iridocyclitis" were reported in the formative literature from France, Austria, Germany, Greece, Netherlands, Japan, Czech Republic, Poland, Belgium and Switzerland over centuries before Dr. Adamantiades, that probably spans from Hippocratic writings in 460–377 BCE,30 through the 18th and 19th centuries,31,32 up to the 20th century,33–39, all of which described recurrent iritis/iridocyclitis with or without hypopyon uveitis. It is clear that all of the presented articles were published before the single case of Dr. Adamantiades.28,29

Having presented the numerous case reports of "recurrent hypopyon iritis/iridocyclitis", let us evaluate now the symptomatic constellations that have also been presented and reported repeatedly before the single case of Dr. Adamantiades. Recurrent mucocutaneous lesions consisting of orogenital aphthous ulcerations were reported independently by Neumann40 and Christlieb.41 Reis33 reported the association between "relapsing ocular inflammation", cutaneous lesions (erythema nodosum) and arthritis in a man. Blüthe34 published the same symptom constellation consisting of "relapsing hypopyon-iridocyclitis", mucocutaneous lesions and arthritis with histological evidences of uveitis and optic nerve atrophy, which was completely identical to the single case of Dr. Adamantiades.28,29 The association between recurrent hypopyon iridocyclitis, recurrent arthritis and skin lesions was also reported by Gilbert between 1920 and 1925,35–38 also clearly and completely identical again with the single case of Dr. Adamantiades.28,29 Planner and Remenowsky42 reported a case with iritis and genital lesions and Weve39 published a case with recurrent hypopyon-iridocyclitis, arthritis, mucocutaneous lesions, periodontitis and neurologic findings, once more of exactly the same symptom constellation as the single case of Dr. Adamantiades.28,29 Shigeta32 from Japan reported a man with recurrent mucocutaneous ulcers and hypopyon iritis with histological detection of uveitis and optic nerve atrophy. Pils43 published a case with mucocutaneous lesions, thrombophlebitis and arthralgia. Grütz44 and Carol and Ruys45 published a total of two patients with recurrent genital lesions and arthralgia. Samek and Fisher46 reported a case with recurrent mucocutaneous lesions and erythema nodosum with the first use of the pathergy test. Walter and Roman47 reported recurrent mucocutaneous lesions with histologically demonstrated leukocytoclastic vasculitis in a genital ulcer and a skin lesion. Kumer48 reported mucocutaneous lesions in women with a histologic detection of leukocytoclastic vasculitis in a genital ulcer and an erythema nodosum lesion with some CNS signs. It is clear that all these symptom and sign constellations were published before the single case of Dr. Adamantiades.28,29

However, the aforementioned papers attributed the symptoms or signs either to another disease such as, for instance, septic metastasis,36–38 tuberculosis,49 and focal bacterial infection or allergy,39 or to a coincidence, and none of them indicated "a new or a single syndrome" with "classical triad" consisting of "triple symptom complex". Obviously, the single case of Dr. Adamantiades28,29 is not different from the above-mentioned articles.

In the following months and years, Dr. Benedictos Adamantiades, an ophthalmologist from Greece, presented and then reported a 20-year-old male patient with recurrent hypopyon iritis, mucocutaneous symptoms and arthritis, as I stated above.28,29 In his paper, Dr. Adamantiades indicated that "recurrent hypopyon iritis", in other words, "Sur un cas d'iritis à hypopyon récidivante" as it appears in the title, constitutes itself a distinct clinical entity (but not "triple symptom complex" of Dr. Behçet). However, "relapsing ocular lesions", in other words, "l'iritis récidivante à hypopyon"50 or "l'uvéite récidivante à hypopyon",51 may occur not only in many ocular or non-ocular diseases, but also in various infectious or non-infectious systemic disorders. Among these diseases, for instance, we can make a list of diseases here such as tuberculosis, syphilis, leprosy, various vasculitides including Vogt–Koyanagi–Harada syndrome and some other endogenous uveitides, as well as staphylococcal bacteremia, sepsis or local bacterial infection, as some of the etiological factors.2 Indeed, Dr. Adamantiades included syphilis, tuberculosis and bacteremia in the etiology and his patient received anti-syphilitic treatment, though the disease was not cured.52–55 He then ascribed the disease to bacterial staphylococcal infection and focal illness, and cited in his paper the similar cases of Reis,33 Gilbert35 and Weve,37 all of which also ascribed the disease to such etiologies. Between 1930 and 1940 until the important publications of Dr. Hulusi Behçet, we can also find numerous articles with the same symptoms or signs constellation. Among these reports, for instance, similar cases with "recurrent ocular lesions" and/or "recurrent mucocutaneous symptoms or signs" were further reported by Dascalopoulos,56 von Hippel,57 Matras,58 Urbanek,59,60 Whitwell,61 Nishimura,62 Blobner,63 Weekers-Reginster,64,65 Knapp66 and Schmidt.67,68

If credit is to be given to the aforesaid authors who reported patients with similar monosymptomatic or identical oligosymptomatic/polysymptomatic manifestations to that of Dr. Adamantiades, albeit attributing the findings to other etiological local or systemic diseases, a new name should be suggested: "Hippocrates-...-Janin-Neumann-Reis-Blüthe-Gilbert-Planner-Remenovsky-Weve-Shigeta-Pils-Grütz-Carol-Ruys-Samek-Fischer-Walter-Roman-Kumer-Urbanek-Adamantiades-Dascalopoulos-von Hippel-Matras-Whitwell-Nishimura-Blobner-Weekers-Reginster-Knapp-Schmidt-Behçet disease", though several others have remained still unnamed here. Therefore, Leibowitz and co-authors should clearly answer the most important question of why should only Dr. Benedictos Adamantiades, among more than 30 aforelisted esteemed authors, be honored?

Therefore, as the International Behçet's Society of the UK strongly indicated, the single case of Dr. Adamantiades was not the first to describe the manifestations of the disease, he did not report or indicate an association as "triple symptom complex" and, more importantly, Dr. Adamantiades, like the previous ancestors, did not recognize the true nature of the disease and was not able to describe it as a distinct entity or syndrome, namely as a "classical triad" consisting of recurrent oral aphthae, genital ulcers and hypopyon uveitis.52–55

Having presented all historical publications with evidence-based articles, let us see what Dr. Hulusi Behçet did for the innovation of this unique disorder. Dr. Behçet was the first author who recognized a characteristic symptom and sign constellation, grouped all the manifestations (recurrent oral aphthae, genital ulcerations, recurrent hypopyon uveitis) himself into a single disease first in 1937,69 and published it in a famous German Journal with a German title "Über rezidivierende, Aphthöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien". Afterwards, he described his further results in 193970 again in German as "Einige Bemerkungen zu meinen Beobachtungen über den Tri-Symptomenkomplex" in another publication and then once more in 1940,71 in English in that case, as "Some observations on the clinical picture of the so-called triple symptom complex", clearly and unquestionably indicating a strong association between three unrelated manifestations together as "a new, specific and completely separate clinical entity or syndrome".

Following these publications of Dr. Behçet, many authors of different racial and ethnic origin in Northern, Southern, Eastern and Western populations started to publish such puzzling cases with "triple symptom complex" consisting of so-called "classical triad" (namely dermatologic, ophthalmic and oro-genital lesions). Among these authors, for instance, Jensen72,73 from Denmark first used the eponym "Behçet" in the title of the paper to describe the "triple symptom complex" of Behçet consisting of "oculo-oromucocutaneous" manifestations, which was described in the title as "Sur les ulcérations aphteuses de la muqueuse de la bouche et de la peau génitale combinées avec les symptômes oculaires (=Syndrôme Behçet)". Similarly, Berlin74 used the eponym "Behçet's syndrome" in the title of the case report to indicate again the components of "triple symptom complex" as "mouth, genital and eye lesions", which was clearly described in the title as "Behçet's syndrome with involvement of central nervous system. Report of a case, with necropsy, of lesions of the mouth, genitalia and eyes; review of the literature". Ephraim75 from Israel first used the "triple symptom complex of Behçet" in the title of the publication. Ollendorff Curth76,77 from the USA then published two papers on American patients and reported the abortive form of Behçet's syndrome, then published the "classical triad" of Dr. Hulusi Behçet in the title of the paper, describing it as "Recurrent genito-oral aphthosis and uveitis with hypopyon (Behçet's syndrome)", indicating once more the "triple symptom complex" of Dr. Behçet. Finally, the eponym "Behçet's disease" was first used and titled by Feigenbaum and Kornblueth,78 and Dorello79 first described the disease as "Dermato-stomato-ophthalmia". Indeed, these papers resulted in the popularization of "Behçet" disease or syndrome in every country that honors the first describer of the "triple symptom complex".

Dr. Hulusi Behçet, as a dermatologist, put particular importance on the recurrent oral ulcerations (aphthosis) that is today the "universal hallmark" and the only sine qua non symptom of this unique disorder according to the International Study Group Criteria for diagnosis of Behçet's disease held in the UK (but not "recurrent iridocyclitis" of Dr. Adamantiades). In turn, "recurrent iridocyclitis with or without hypopyon uveitis", stressed by Dr. Adamantiades from the ophthalmologist's perspective as a clinical entity, is encountered in approximately half of Behçet patients and the diagnosis of Behçet disease can still be made even if the patient has no such an ocular involvement.1–4,14–22

Having been confronted with all these publications, physicians and dermatologists came together in Europe and, at the suggestion of Professor Mischner of the Zurich Medical Faculty, the disease was named "Morbus Behçet" during the International Medical Congress of Geneva in Switzerland in 1947. Afterwards the rate of publications on this unique disorder accelerated in various languages (Japanese, German, French, Hungarian, Italian, Russian, Czech, English, Spanish) and countless authors from around the world have begun to report articles in which the disease is named as either "triple symptom complex",80–84 "Behçet disease",85–88 or "Behçet syndrome"89–94 until the death of Dr. Benedictos Adamantiades in 1962.

If Dr. Adamantiades had really worried about the use of the eponym "Behçet" in the titles of these articles in the last century, I think he might himself have written at least a "letter to the editor" when he was alive (1875–1962) not only to the aforementioned international medical journals, as I do today, but also to at least one of the following authors who named the disease as "Behçet syndrome", "Behçet disease" or "triple symptom complex of Behçet" as well as to both the "International Dermatology Society" who honored the first describer of "Tri-Symptomenkomplex/Triple Symptom Complex" and Professor Mischner of the Zurich Medical Faculty who suggested "Morbus Behçet" to credit the first describer of "classical triad" in 1947.

In opposition to Leibowitz et al., Dr. Adamantiades had really realized that it was Dr. Hulusi Behçet who indicated a new and a separate syndrome with "triple symptom complex", as we easily understand from the title of one of Dr. Adamantiades’ subsequent papers published just 12years after the original report of Dr. Behçet (Adamantiades B, Lorando N. Sur le syndrome complexe de uvéite récidivante ou soi-distant syndrome complexe de Behçet. Presse Med 1949;57:501). On the other hand, Dr. Adamantiades, as an ophthalmologist, was so convinced that he had discovered a distinct clinical entity characterized by "recurrent ocular lesions", he published more and more papers on the same subject even before and after the worldwide recognition and acceptance of the disease as "Morbus Behçet" in 1947, and insistently and repeatedly concentrated on "relapsing hypopyon iritis" during the following years, as his titles clearly disclose in French,50,51 Greek and English until 1958.95

In the light of both above-mentioned evidence-based historical articles over centuries, I completely disagree with Leibowitz et al. and, therefore, strongly reject their unproved, non-referenced and confusing disease naming as it appears in their title and throughout the text. All classical textbooks of dermatology, rheumatology and ophthalmology and any other sections or subheadings of medical books title this entity as "Behçet disease" or "Behçet syndrome", not as "Adamantiades–Behçet disease". Likewise, international symposiums, congresses, courses and conferences as well as the American Behçet's Disease Association (ABDA), the International Society for Behçet's Disease (ISBD) of UK, the Japanese Behçet's Disease Research Committee, the French Behçet's Association, the Italian Behçet's Disease Association, the Korean Behçet's Association, the Behçet Israel Group (BIG) and finally the Turkish Ophthalmology Behçet Society (TOD) strongly suggest and use the eponym "Behçet", not "Adamantiades–Behçet disease" or ABD.

Authors who know historical evidence deeply enough use the eponym "Behçet" without exception. Esteemed investigators of the given journal should follow the instructions of the American Behçet's Disease Association and the International Behçet's Society that acknowledges the origin of Behçet disease on their official web sites. I think it is time to stay away from unrealistic chaotic naming of a known disease that results in needless and unproductive discussions, as well as to keep away from misleading the innocent readers of medical journals by using arbitrary new terms in the articles for the definition of established diseases after more than 65years have elapsed since its first innovation and subsequent worldwide acceptance. In other words, it is the responsibility of the authors and editors to inform the readers of the journals with settled and approved knowledge of science and eponyms. Moreover, we should be respectful to all faithful authors for their diligent efforts in the past who had papers using the eponym "Behçet" in more than 6000 articles on Medline/PubMed search with thousands of citations over decades. The authors of the present article should also remember that Dr. Benedictos Adamantiades himself called the disease "Behçet" in his paper just 12years after the initial reports of Dr. Hulusi Behçet between 1937 and 1940.


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