Introduction: Dedicated Mondays and an Acquaintance-Based View of Behçet Syndrome

  • Hasan YaziciEmail author


Behçet syndrome (BS) is a complex construct of unknown etiology(ies). As such a splitting rather than a lumping approach should be used in recognizing BS and deciphering its yet unknown disease mechanisms. So instead of a universal, we should have subspecialty-based classification/diagnostic criteria sets. Similarly, we should more aim to understand how it differs from other inflammatory diseases of unknown etiology instead of going at lengths to show how it is like these conditions, an attempt best exemplified by the recent concept of MHC-I-opathy.


Lumping Splitting Subspecialty-based criteria MHC-I-opathy 


  1. 1.
    O’Duffy JD. Suggested criteria for diagnosis of Behcet’s disease. J Rheumatol. 1974;1(Suppl 1):Abstr. 32:18.Google Scholar
  2. 2.
    International Study Group for Behçet Disease. Criteria for diagnosis of Behçetdisease. Lancet. 1990;335:1078–80.Google Scholar
  3. 3.
    Hunder GG. The use and misuse of classification and diagnostic criteria for complex diseases. Ann Intern Med. 1998;129:417–8.CrossRefGoogle Scholar
  4. 4.
    Yazici H, Yazici Y. Disease classification/diagnosis criteria. In: Yazici H, Yazici Y, Lesaffre E, editors. Understanding evidence-based rheumatology. New York: Springer; 2014. p. 73.Google Scholar
  5. 5.
    Aggarwal R, Ringold S, Khanna D, et al. Distinctions between diagnostic and classification criteria. Arthritis Care Res (Hoboken). 2015;67:891–7.CrossRefGoogle Scholar
  6. 6.
    Mørland B, Brantsæter AB, Fuglesang JE, et al. Effect of oseltamivir (Tamiflu®) for the prevention and treatment of influenza during an influenza pandemic [Internet]. Oslo, Norway: Knowledge Centre for the Health Services at The Norwegian Institute of Public Health (NIPH); 2005. Report from Norwegian Knowledge Centre for the Health Services (NOKC) No. 01-2005. Accessed 23 Apr 2019.Google Scholar
  7. 7.
    Fries JF, Hochberg MC, Medsger TA Jr, et al. Criteria for rheumatic disease: different types and different functions. Arthritis Rheum. 1994;37:454–62.CrossRefGoogle Scholar
  8. 8.
    Yazici H, Seyahi E, Yurdakul S. Behçet syndrome is not so rare: why do we need to know? Arthritis Rheum. 2008;58:3640–3.CrossRefGoogle Scholar
  9. 9.
    Rodriguez A, Calonge M, Pedroza-Seres M, et al. Referral patterns of uveitis in a tertiary eye care center. Arch Ophthalmol. 1996;114:593–9.CrossRefGoogle Scholar
  10. 10.
    Goto H, Mochizuki M, Yamaki K, et al. Epidemiological survey of intraocular inflammation in Japan. Jpn J Ophthalmol. 2007;51:41–4.CrossRefGoogle Scholar
  11. 11.
    Tugal-Tutkun I, Onal S, Ozyazgan Y, Soylu M, Akman M. Validity and agreement of uveitis experts in interpretation of ocular photographs for diagnosis of Behçet uveitis. Ocul Immunol Inflamm. 2013;22:461–8.CrossRefGoogle Scholar
  12. 12.
    Moll JM, Haslock I, Macrae IF, et al. Associations between ankylosing spondylitis, psoriatic arthritis, Reiter’s disease, the intestinal arthropathies and Behcet’s syndrome. Medicine (Baltimore). 1974;53:343–64.CrossRefGoogle Scholar
  13. 13.
    Yazici H. Behçet hastalığı seronegatif spondartritlerden midir? Dozent’s thesis. University of Istanbul. 1978.Google Scholar
  14. 14.
    Krause I, Leibovici L, Guedj D, et al. Disease patterns of patients with Behçet disease demonstrated by factor analysis. Clin Exp Rheumatol. 1999;17:347–50.PubMedGoogle Scholar
  15. 15.
    Tunc R, Keyman E, Melikoglu M, et al. Target organ associations in Turkish patients with Behçet disease: a cross sectional study by exploratory factor analysis. J Rheumatol. 2002;29:2393–6.PubMedGoogle Scholar
  16. 16.
    Diri E, Mat C, Hamuryudan V, Yurdakul S, Hizli N, Yazici H. Papulopustular skin lesions are seen more frequently in patients with Behçet syndrome who have arthritis: a controlled and masked study. Ann Rheum Dis. 2001;60:1074–6.CrossRefGoogle Scholar
  17. 17.
    Hatemi G, Bahar H, Uysal S, et al. The pustular skin lesions in Behçet syndrome are not sterile. Ann Rheum Dis. 2004;63:1450–2.CrossRefGoogle Scholar
  18. 18.
    Hatemi G, Fresko I, Tascilar K, Yazici H. Enthesopathy is increased among Behçet syndrome patients with acne and arthritis: an ultrasonographic study. Arthritis Rheum. 2008;58:1539–45.CrossRefGoogle Scholar
  19. 19.
    Karaca M, Hatemi G, Sut N, Yazici H. The papulopustular lesion/arthritis cluster of Behçet’s syndrome also clusters in families. Rheumatology (Oxford). 2012;51:1053–60.CrossRefGoogle Scholar
  20. 20.
    Priori R, Ceccarelli F, Milani S, et al. Do Behçet syndrome patients with acne and arthritis comprise a true subset? Comment on the article by Hatemi et al. Arthritis Rheum. 2009;60:1201–2.CrossRefGoogle Scholar
  21. 21.
    Tunc R, Saip S, Siva A, Yazici H. Cerebral venous thrombosis is associated with major vessel disease in Behçet syndrome. Ann Rheum Dis. 2004;63:1693–4.CrossRefGoogle Scholar
  22. 22.
    Yazici H, Fresko I, Yurdakul S. Behçet syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol. 2007;3:148–55.CrossRefGoogle Scholar
  23. 23.
    Tascilar K, Melikoglu M, Ugurlu S, et al. Vascular involvement in Behçet’s syndrome: a retrospective analysis of associations and the time course. Rheumatology. 2014;53:2018–22.CrossRefGoogle Scholar
  24. 24.
    McGonagle D, Aydin SZ, Gul A, Mahr A, Direskeneli H. “MHC-I-opathy”—unified concept for spondyloarthritis and Behçet disease. Nat Rev Rheumatol. 2015;11:731–40.CrossRefGoogle Scholar
  25. 25.
    Yazici H, Seyahi E, Hatemi G, Yazici Y. Behçet syndrome: a contemporary view. Nat Rev Rheumatol. 2018;14:107–19.CrossRefGoogle Scholar
  26. 26.
    McKusick VA. The Gordon Wilson Lecture: the clinical legacy of Jonathan Hutchinson (1828–1913): syndromology and dysmorphology meet genomics. Trans Am Clin Climatol Assoc. 2005;116:15–38.PubMedPubMedCentralGoogle Scholar
  27. 27.
    Gül A. Behçet disease as an autoinflammatory disorder. Curr Drug Targets Inflamm Allergy. 2005;4:81–3.CrossRefGoogle Scholar
  28. 28.
    Yazici H, Fresko I. Behçet disease and other autoinflammatory conditions: what’s in a name? Clin Exp Rheumatol. 2005;23.(4 Suppl 38:S1–2.PubMedGoogle Scholar
  29. 29.
    Seyahi E, Ugurlu S, Cumali R, et al. Atherosclerosis in Behçet syndrome. Semin Arthritis Rheum. 2008;38:1–12.CrossRefGoogle Scholar
  30. 30.
    Ambrose N, Pierce IT, Gatehouse PD, Haskard DO, Firmin DN. Magnetic resonance imaging of vein wall thickness in patients with Behçet’s syndrome. Clin Exp Rheumatol. 2014;32(4 Suppl 84):S99–102.PubMedGoogle Scholar
  31. 31.
    Seyahi E, Gjoni M, Durmaz ES, et al. Increased vein wall thickness in Behçet syndrome. J Vasc Surg. 2019;in press. Scholar
  32. 32.
    Alibaz-Oner F, Ergelen R, Mutis A, et al. Venous vessel wall thickness in lower extremity is increased in male Behcet’s disease patients. Abs. no. O09-P084. In: 18th international congress on Behçet disease. Rotterdam; 2018.Google Scholar
  33. 33.
    Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77:808–18.PubMedGoogle Scholar
  34. 34.
    Kural-Seyahi E, Fresko I, Seyahi N, et al. The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003;82:60–76.CrossRefGoogle Scholar
  35. 35.
    Benezra D, Cohen E. Treatment and visual prognosis in Behçet disease. Br J Ophthalmol. 1986;70:589–92.CrossRefGoogle Scholar
  36. 36.
    Hamuryudan V, Seyahi E, Ugurlu S, et al. Pulmonary artery involvement in Behçet’s syndrome: effects of anti-Tnf treatment. Semin Arthritis Rheum. 2015;45:369–73.CrossRefGoogle Scholar
  37. 37.
    Kurtuncu M, Tuzun E, Mutlu M, et al. Clinical patterns and course of neuro-Behçet disease: analysis of 354 patients comparing cases presented before and after 1990. Clin Exp Rheumatol. 2008;26(4 Suppl 50):17.Google Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of MedicineAcademic HospitalIstanbulTurkey

Personalised recommendations