Jornal Vascular Brasileiro
Jornal Vascular Brasileiro
Letter to the Editor

HLA-genotyping and the historical naming process of an old disorder

Genotipagem de HLA e o processo histórico de nomenclatura de uma doença antiga

Cem Evereklioglu

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Dear Editor,

I read the article by Metzger et al. entitled “Endovascular treatment of aortic saccular aneurysms associated with Adamantiades-Behçet disease” (BD, 2021). They reported a 49-year-old man with an abdominal aorta aneurysm treated with an inferior vena cava filter and endovascular repair. I thank the authors for their efforts. However, I have 2 questions to be clarified:

1) They stated that BD has associations with HLA-B51 and HLA-B27. However, BD is strongly associated with HLA-B51,1,2 but weakly with HLA-B273 and there is no study linking BD strongly with HLA-B27. Indeed, an HLA-B27-related non-infectious entity is associated with ankylosing spondylitis uveitis.4,5 I wonder whether the authors performed HLA analyses or did they demonstrate any link with HLA-B27 or HLA-B51?

2) This disorder was called by the authors “Adamantiades-Behçet”. Investigators before Hulusi Behçet reported several Behçet findings since Hippocrates,6 such as Planner and Remenovsky (1922)7 and Pils (1925).8 However, none indicated a novel disease with the “classical-triad”. Adamantiades concluded that “hypopyon iritis” (not classical-triad) constitutes a distinct entity, which can occur in many disorders.9 Hulusi Behçet was the first to realize and group dermato-ophthalmological findings into one disease (triple-symptom-complex; 1937-1940).10-12 Metzger et al. used the “International Study Group Criteria for BD” (not Adamantiades-Behçet). The authors should respect the name of an established old disease and no-one should try to change it. Therefore, the authors should address the reason for preferring the eponym Adamantiades-Behçet. Why should only Dr. Adamantiades be honored? PubMed reveals 13,315 Behçet articles (1946-2022), and 175 Adamantiades-Behçet (1970-2022). Similarly, they used 17 articles in the reference and only one paper stated it as “Adamantiades-Behçet”. It is the authors’ responsibility to use the correct, accepted, and established international eponym because editors may not realize such a historical naming process. The “Jornal Vascular Brasileiro” published 5 articles, all using Behçet without exception. Moreover, the American BD Association, International BD Society, and Japan BD Committee all call the disease “Behçet” to honor the first describer of the “triple-symptom-complex”. Furthermore, textbooks of dermatology, rheumatology, and ophthalmology, and international congresses call this entity BD, not Adamantiades-Behçet. Adamantiades himself named the syndrome “Behçet”.13 I think it is time to abandon efforts to change the name of an old syndrome.


1 Evereklioglu C. Current concepts in the etiology and treatment of Behçet disease. Surv Ophthalmol. 2005;50(4):297-350. PMid:15967189.

2 Leccese P, Alpsoy E. Behçet’s disease: an overview of etiopathogenesis. Front Immunol. 2019;10:1067. PMid:31134098.

3 Khabbazi A, Vahedi L, Ghojazadeh M, Pashazadeh F, Khameneh A. Association of HLA-B27 and Behcet’s disease: a systematic review and meta-analysis. Auto Immun Highlights. 2019;10(1):2. PMid:30891643.

4 Jung JH, Bang CH, Seok H, Choi SJ, Song GG. Clinical findings of ankylosing spondylitis with and without Human Leukocyte Antigen (HLA)-B27 and HLA-B51. Ann Acad Med Singapore. 2019;48(10):321-9. PMid:31875469.

5 Sakly N, Boumiza R, Zrour-Hassen S, et al. HLA-B27 and HLA-B51 determination in Tunisian healthy subjects and patients with suspected ankylosing spondylitis and Behçet’s disease. Ann N Y Acad Sci. 2009;1173(1):564-9. PMid:19758200.

6 Evereklioglu C. Behçet’s disease or Adamantiades-Behçet disease? An evidence-based historical survey. Med Sci Monit. 2010;16(6):RA136-42. PMid:20512105.

7 Planner H, Remenovsky F. Beitrage zur Kenntnis der Ulcerationen am außeren weiblichen Genitale. Arch Dermatol Syphil. 1922;140(1):162-88.

8 Pils H. Ein Beitrag zur Aphthosis. Arch Dermatol Syphil. 1925;149(1):4-8.

9 Adamantiades B. A case of recurrent hypopyon iritis. Proc Med Soc Athens. 1930;2:586-93.

10 Behçet H. Über rezidivierende, Aphthöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatol Wochenschr. 1937;105:1152-7.

11 Behçet H. Einige Bemerkungen zu meinen Beobachtungen über den Tri-Symptomenkomplex. Med Welt. 1939;13:1222-7.

12 Behçet H. Some observations on the clinical picture of the so-called triple symptom complex. Dermatologica. 1940;81(2):73-83.

13 Adamantiades B, Lorando N. Sur le syndrome complexe de uvéite récidivante ou soi-distant syndrome complexe de Behçet. Presse Med. 1949;57(36):501-3. PMid:18152604.

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