Skip to main content
Log in

Anmerkungen zur Pathogenese des Morbus Behçet

Ein Schlüssel zum Verständnis neuer Therapien?

Comments on the pathogenesis of Behçet’s disease

A key to understanding new therapies?

  • Leitthema
  • Published:
Der Ophthalmologe Aims and scope Submit manuscript

Zusammenfassung

Die Ätiologie und Pathogenese des Behçet-Syndroms (Morbus Behçet) sind bisher nicht geklärt. Postuliert wird eine multifaktorielle Genese, die – ausgelöst durch ein möglicherweise infektiöses Agens – bei Vorliegen einer genetischen Disposition (HLA-B51) zu einer generalisierten Vaskulitis führt. Eine überschießende Reaktion des angeborenen, später auch erworbenen Immunsystems u. a. gegen „Heat-Shock-Proteine“ schließt sich vermutlich sekundär an. In diesem Beitrag wird eine kurze Zusammenfassung jüngster Ergebnisse gegeben. Sie sollen v. a. im Hinblick auf neue therapeutische Möglichkeiten und klinisch relevante Perspektiven dargestellt werden.

Abstract

The etiology and pathogenesis of Behçet’s syndrome remains unclear. It has been postulated that an infectious agent may induce an immune-mediated, generalized vasculitis in genetically predisposed individuals (HLA-B51 +). There is accumulating evidence that an abnormality of the innate and adaptive immune response, e.g. directed against heat shock proteins plays an important role in Behçet’s syndrome. This article is intended to evaluate the more recent knowledge on this disorder and evaluate new therapeutic options.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Abb. 1
Abb. 2

Literatur

  1. Aki T, Karincaoglu Y, Seyhan M, Batcioglu K (2006) Serum substance P and calcitonin gene-related peptide levels in Behçet’s disease and their association with disease activity. Clin Exp Dermatol 3:583–587

    Article  Google Scholar 

  2. Alexander T, Klotz O, Feist E et al (2005) Successful treatment of acute visual loss in Muckle-Wells syndrome with interleukin 1receptor antagonist. Ann Rheum Dis 64:1245–1246

    Article  PubMed  CAS  Google Scholar 

  3. Amadi-Obi A, Yu CR, Liu X et al (2007) TH17 cells contribute to uveitis and scleritis and are expanded by IL-2 and inhibited by IL-27/STAT1. Nat Med 13:711–718

    Article  PubMed  CAS  Google Scholar 

  4. Aydin E, Sögüt S, Ozyurt H et al (2004) Comparison of serum nitric oxide, malondialdehyde levels, and antioxidant enzyme activities in Behçet’s disease with and without ocular disease. Ophthalmic Res 36:177–182

    Article  PubMed  CAS  Google Scholar 

  5. Ben Ahmed M, Houman H, Miled M et al (2004) Involvement of chemokines and Th1 cytokines in the pathogenesis of mucocutaneous lesions of Behçet’s disease. Arthritis Rheum 50:2291–2295

    Article  Google Scholar 

  6. Botsios C, Sfriso P, Furlan A et al (2008) Resistant Behçet disease responsive to anakinra. Ann Intern Med 149:284–286

    PubMed  Google Scholar 

  7. Calguneri M, Kiraz S, Ertenli I et al (1996) The effect of prophylactic penicillin treatment on the course of arthritis episodes in patients with Behcet’s disease. A randomized clinical trial. Arthritis Rheum 39:2062–2065

    Article  PubMed  CAS  Google Scholar 

  8. Dinarello CA (2009) Immunological and inflammatory functions of the interleukin-1 family. Annu Rev Immunol 27:519–550

    Article  PubMed  CAS  Google Scholar 

  9. Direskeneli H (2001) Behçet’s disease: infectious aetiology, new autoantigens, and HLA-B51. Ann Rheum Dis 60:996–1002

    Article  PubMed  CAS  Google Scholar 

  10. Direskeneli H (2006) Autoimmunity vs autoinflammation in Behcet’s disease: Do we oversimplify a complex disorder? Rheumatology 45:1461–1465

    Article  PubMed  CAS  Google Scholar 

  11. Ekşioglu-Demiralp E, Kibaroglu A, Direskeneli H et al (1999) Phenotypic characteristics of B cells in Behçet’s disease: increased activity in B cell subsets. J Rheumatol 26:826–832

    PubMed  Google Scholar 

  12. Ertenli I, Kiraz S, Calguneri M et al (2001) Synovial fluid cytokine levels in Behçet’s disease. Clin Exp Rheumatol 19:37–41

    Article  Google Scholar 

  13. Gadola SD (2009) Interleukin-1 cytokines, inflammasomes, NOD-signalosomes and autoinflammation. Z Rheumatol 68:712–719

    Article  PubMed  CAS  Google Scholar 

  14. Geri G, Terrier B, Rosenzwajg M et al (2011) Critical role of IL-21 in modulating TH17 and regulatory T cells in Behçet disease. J Allergy Clin Immunol 128:655–664

    Article  PubMed  CAS  Google Scholar 

  15. Glaser RL, Goldbach-Mansky R (2008) The spectrum of monogenic autoinflammatory syndromes: understanding disease mechanisms and use of targeted therapies. Curr Allergy Asthma Rep 8:288–298

    Article  PubMed  CAS  Google Scholar 

  16. Gül A, Uyar FA, Inanc M et al (2001) Lack of association of HLA-B*51 with a severe disease course in Behcet’s disease. Rheumatology 40:668–672

    Article  PubMed  Google Scholar 

  17. Gür-Toy G, Lenk N, Yalcin B et al (2005) Serum interleukin-8 as a serologic marker of activity in Behçet’s disease. Int J Dermatol 44:657–660

    Article  PubMed  Google Scholar 

  18. Hamzaoui K, Hamzaoui A, Bouajina L, Houman H (2005) Circulating soluble CD28 in patients with Behçet’s disease: relationship to clinical manifestations. Clin Exp Rheumatol 23(4 Suppl 38):49–52

    Google Scholar 

  19. Hawkins PN, Lachmann HJ, McDermott MF (2003) Interleukin-1-receptor antagonist in the Muckle-Wells syndrome. N Engl J Med 348:2583–2584

    Article  PubMed  Google Scholar 

  20. Hirohata S, Hashimoto T (1998) Abnormal T cell responses to bacterial superantigens in Behçet’s disease (BD). Clin Exp Immunol 112:317–324

    Article  PubMed  CAS  Google Scholar 

  21. Hoffman HM, Rosengren S, Boyle DL et al (2004) Prevention of cold-associated acute inflammation in familial cold autoinflammatory syndrome by interleukin-1 receptor antagonist. Lancet 364:1779–1785

    Article  PubMed  CAS  Google Scholar 

  22. Hu W, Hasan A, Wilson A et al (1998) Experimental mucosal induction of uveitis with the 60-kDa heat shock protein-derived peptide 336–351. Eur J Immunol 28:2444–2455

    Article  PubMed  CAS  Google Scholar 

  23. Kim TW, Chung H, Yu HG (2010) Chemokine expression of intraocular lymphocytes in patients with Behçet uveitis. Ophthalmic Res 45:5–14

    Article  PubMed  Google Scholar 

  24. Mariathasan S, Weiss DS, Newton K et al (2006) Cryopyrin activates the inflammasome in response to toxins and ATP. Nature 440:228–232

    Article  PubMed  CAS  Google Scholar 

  25. Martinon F, Mayor A, Tschopp J (2009) The inflammasomes: guardians of the body. Annu Rev Immunol 27:229–265

    Article  PubMed  CAS  Google Scholar 

  26. Mizuki N, Meguro A, Ota M et al (2010) Genome-wide association studies identify IL23R-IL12RB2 and IL10 as Behçet’s disease susceptibility loci. Nat Genet 42:703–706

    Article  PubMed  CAS  Google Scholar 

  27. Stanford M, Whittall T, Bergmeier LA et al (2004) Oral tolerization with peptide 336–351 linked to cholera toxin B subunit in preventing relapses of uveitis in Behcet’s disease. Clin Exp Immunol 137:201–208

    Article  PubMed  CAS  Google Scholar 

  28. Verity DH, Wallace GR, Vaughan RW et al (1999b) HLA and tumour necrosis factor (TNF) polymorphisms in ocular Behcet’s disease. Tissue Antigens 54:264–272

    Article  PubMed  CAS  Google Scholar 

  29. Zamecki KJ, Jabs DA (2010) HLA typing in uveitis: use and misuse. Am J Ophthalmol 149:189–193

    Article  PubMed  CAS  Google Scholar 

  30. Zouboulis CC, Buttner P, Djawari D et al (1993) The HLA pattern in Adamantiades-Behcet’s disease in Germany. Association of occurrence, clinical symptoms and follow-up in 39 patients. Hautarzt 44:81–85

    PubMed  CAS  Google Scholar 

Download references

Interessenkonflikt

Keine Angaben.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to U. Pleyer.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Pleyer, U., Hazirolan, D. & Stübiger, N. Anmerkungen zur Pathogenese des Morbus Behçet. Ophthalmologe 109, 563–567 (2012). https://doi.org/10.1007/s00347-012-2586-z

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00347-012-2586-z

Schlüsselwörter

Keywords

Navigation