PDF herunterladen
Klin Monbl Augenheilkd 2020; 237(05): 591-598
DOI: 10.1055/a-1140-3295
Übersicht
Georg Thieme Verlag KG Stuttgart · New York

Uveitis bei Behçet-Syndrom in Deutschland – Prognose unter aktueller Therapie

Uveitis in Behçetʼs Syndrome in Germany – Prognosis During Current Therapy
Nicole Stübiger
1   Klinik und Poliklinik für Augenheilkunde, Universitätsklinikum Hamburg-Eppendorf
,
Christoph Deuter
2   Department für Augenheilkunde, Universitätsklinikum Tübingen
,
Deshka Doycheva
2   Department für Augenheilkunde, Universitätsklinikum Tübingen
› Institutsangaben
Weitere Informationen

Publikationsverlauf

eingereicht 26. Februar 2020

akzeptiert 17. März 2020

Publikationsdatum:
20. Mai 2020 (online)

Auch verfügbar auf
    Lizenzen und Reprints

Zusammenfassung

Das Behçet-Syndrom (BS) ist eine entzündliche Systemerkrankung, die mit multiplen Organmanifestationen einhergeht. Häufigste Krankheitssymptome sind orale Aphthen, genitale Ulzerationen, Hauteffloreszenzen und Augenmanifestationen. Die Diagnose des Behçet-Syndroms wird anhand klinischer Symptome gestellt. Typische okuläre Präsentation beim Behçet-Syndrom ist die rezidivierende nicht granulomatöse Panuveitis mit okklusiver retinaler Vaskulitis. Früher waren systemische Steroide und konventionelle Immunsuppressiva die einzige therapeutische Option beim Behçet-Syndrom. Unter dieser Therapie konnte jedoch häufig keine stabile Remission der Augenbeteiligung erreicht werden und die Prognose der Behçet-Uveitis, insbesondere bei Vorliegen einer okklusiven retinalen Vaskulitis, galt lange Zeit als äußerst schlecht. In den letzten Jahren hat die Einführung der Biologika die Therapie des Behçet-Syndroms revolutioniert und die Visusprognose der Patienten deutlich verbessert.

Abstract

Behçetʼs syndrome (BS) is an inflammatory systemic disease associated with multiple organ manifestations. Major symptoms include oral and genital ulcerations, skin lesions and uveitis. Diagnosis of Behçetʼs syndrome is based on clinical findings. A typical for ocular Behçetʼs syndrome is recurrent non-granulomatous panuveitis with occlusive retinal vasculitis. Treatment used to be based only on the use of systemic corticosteroids and conventional immunosuppressives, but most often this treatment did not appear to be sufficient. In consequence, prognosis of ocular BS was very poor over a long period of time – especially if occlusive retinal vasculitis was present. In recent years, the introduction of biologicals has revolutionised the therapeutic regime and consecutively visual prognosis of ocular Behçetʼs syndrome has dramatically improved.

Schlüsselwörter

Behçet-Syndrom - Diagnosekriterien - Uveitis - Therapie - Interferon - TNF-α-Blocker

Key words

Behçet syndrome - diagnostic criteria - uveitis - therapy - interferon - TNF-α blocker
 

  • Literatur

  • 1 Behçet H. Über rezidivierende, aphthöse, durch ein Virus verursachte Geschwüre im Mund, am Auge und an den Genitalien. Dermatol Wochenschr 1937; 105: 1152-1157
    PubMedGoogle Scholar
  • 2 Deuter CME, Kötter I, Wallace GR. et al. Behçetʼs disease: ocular effects and treatment. Prog Retin Eye Res 2008; 27: 111-136
    CrossrefPubMedGoogle Scholar
  • 3 Deuter CM, Zierhut M, Möhle A. et al. Long-term remission after cessation of interferon-α treatment in patients with severe uveitis due to Behçetʼs disease. Arthritis Rheum 2010; 62: 2796-2805
    CrossrefPubMedGoogle Scholar
  • 4 Kötter I, Xenitidis T, Fierlbeck G. et al. Morbus Behçet. Z Rheumatol 2012; 71: 685-697
    CrossrefPubMedGoogle Scholar
  • 5 De Menthon M, Lavalley MP, Maldini C. et al. HLA-B51/B5 and the risk of Behçetʼs disease: a systematic review and meta-analysis of case-control genetic association studies. Arthritis Rheum 2009; 61: 1287-1296
    CrossrefPubMedGoogle Scholar
  • 6 Leccese P, Alpsoy E. Behçetʼs disease: an overview of etiopathogenesis. Front Immunol 2019; 10: 1067
    CrossrefPubMedGoogle Scholar
  • 7 Kneifel CE, Köhler AK, Altenburg A. et al. Epidemiologie der Augenbeteiligung bei Morbus Adamantiades-Behçet. Ophthalmologe 2012; 109: 542-547
    CrossrefPubMedGoogle Scholar
  • 8 Altenburg A, Mahr A, Maldini C. et al. Epidemiologie und Klinik des Morbus Adamantiades-Behçet in Deutschland. Ophthalmologe 2012; 109: 531-541
    CrossrefPubMedGoogle Scholar
  • 9 Sakane T, Takeno M, Suzuki N. et al. Behçetʼs disease. N Engl J Med 1999; 341: 1284-1291
    CrossrefPubMedGoogle Scholar
  • 10 Bang D, Hur W, Lee ES. et al. Prognosis and clinical relevance of recurrent oral ulceration in Behçetʼs disease. J Dermatol 1995; 22: 926-929
    CrossrefPubMedGoogle Scholar
  • 11 Yurdakul S, Yazici H, Tuzun Y. et al. The arthritis of Behçetʼs disease: a prospective study. Ann Rheum Dis 1983; 42: 505-515
    CrossrefPubMedGoogle Scholar
  • 12 Kasahara Y, Tanaka S, Nishino M. et al. Intestinal involvement in Behçetʼs disease: review of 136 surgical cases in the Japanese literature. Dis Colon Rectum 1981; 24: 103-106
    CrossrefPubMedGoogle Scholar
  • 13 Al-Araji A, Kidd DP. Neuro-Behçetʼs disease: epidemiology, clinical characteristics, and management. Lancet Neurol 2009; 8: 192-204
    CrossrefPubMedGoogle Scholar
  • 14 Ozyazgan Y, Ucar D, Hatemi G. et al. Ocular involvement of Behçetʼs syndrome: a comprehensive review. Clin Rev Allerg Immunol 2015; 49: 298-306
    CrossrefPubMedGoogle Scholar
  • 15 Tugal-Tutkun I. Behçetʼs uveitis. Middle East Afr J Ophthalmol 2009; 16: 219-224
    PubMedGoogle Scholar
  • 16 Tugal-Tutkun I, Onal S, Altan-Yaycioglu R. et al. Uveitis in Behçet disease: an analysis of 880 patients. Am J Ophthalmol 2004; 138: 373-380
    CrossrefPubMedGoogle Scholar
  • 17 Tugal-Tutkun I, Gupta V, Cunningham ET. Differential diagnosis of Behçet uveitis. Ocul Immunol Inflamm 2013; 21: 337-350
    CrossrefPubMedGoogle Scholar
  • 18 Tugal-Tutkun I, Onal S, Altan-Yaycioglu R. et al. Neovascularization of the optic disc in Behçetʼs disease. Jpn J Ophthalmol 2006; 50: 256-265
    CrossrefPubMedGoogle Scholar
  • 19 International Study Group for Behçetʼs Disease. Criteria for diagnosis of Behçetʼs disease. Lancet 1990; 335: 1078-1080
    PubMedGoogle Scholar
  • 20 Mason RM, Barnes CG. Behçetʼs syndrome with arthritis. Ann Rheum Dis 1969; 28: 95-103
    CrossrefPubMedGoogle Scholar
  • 21 OʼDuffy JD. Suggested criteria for diagnosis of Behçetʼs disease. J Rheumatol 1974; 1: 18
    PubMedGoogle Scholar
  • 22 Dilsen N, Konice M, Aral O. Our diagnostic Criteria of Behçetʼs Disease – an Overview. In: Lehner T, Barnes CG. eds. Recent Advances in Behçetʼs Disease. London: Royal Society of Medicine Services; 1985: 177-180
    Google Scholar
  • 23 Davatchi D, Assaad-Khalil S, Calamia KT. et al. International Team for the Revision of the International Criteria for Behçetʼs Disease (ITR-ICBD). The International Criteria for Behçetʼs Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol 2014; 28: 338-347
    CrossrefPubMedGoogle Scholar
  • 24 Kötter I, Stübiger N, Zierhut M. Use of Interferon-alpha in Behçetʼs Disease. In: Zierhut M, Ohno S. eds. Immunology of Behçetʼs Disease. Lisse, Netherlands: Swets & Zeitlinger; 2003: 155-159
    Google Scholar
  • 25 Hazirolan D, Stübiger N, Pleyer U. Light on the horizont: biologicals in Behçet uveitis. Acta Ophthalmol 2013; 91: 297-306
    CrossrefPubMedGoogle Scholar
  • 26 Stübiger N. Morbus Behçet. In: Pleyer U. Hrsg. Entzündliche Augenerkrankungen. Berlin: Springer; 2014: 460-469
    Google Scholar
  • 27 Kötter I, Stübiger N, Deuter C. Ocular involvement in rheumatoid arthritis, connective tissue diseases and vasculitis. Z Rheumatol 2017; 76: 673-681
    CrossrefPubMedGoogle Scholar
  • 28 Pleyer U, Stübiger N, Kötter I. Behçet-Syndrom. Gefässchirurgie 2019; 24: 144-151
    CrossrefPubMedGoogle Scholar
  • 29 Hatemi G, Seyahi E, Fresko I. et al. One year in review 2019: Behçetʼs syndrome. Clin Exp Rheumatol 2019; 37: 3-17
    PubMedGoogle Scholar
  • 30 Hatemi G, Christensen R, Bang D. et al. 2018 update of the EULAR recommendations for the management of Behçetʼs syndrome. Ann Rheum Dis 2018; 77: 808-818
    PubMedGoogle Scholar
  • 31 Yazici H, Pazarli H, Barnes CG. et al. A controlled trial of azathioprine in Behçetʼs syndrome. N Engl J Med 1990; 322: 281-285
    CrossrefPubMedGoogle Scholar
  • 32 Adler YD, Mansmann U, Zouboulis CC. Mycophenolate mofetil is ineffective in the treatment of mucocutaneous Adamantiades-Behçetʼs disease. Dermatology 2001; 203: 322-324
    CrossrefPubMedGoogle Scholar
  • 33 Atienza-Mateo B, Martin-Varillas JL, Calvo-Rio V. et al. Comparative study of infliximab versus adalimumab in refractory uveitis due to Behçetʼs disease. National multicenter study of 177 cases. Arthritis Rheumatol 2019; 71: 2081-2089
    PubMedGoogle Scholar
  • 34 Stübiger N, Zierhut M. Interferon alpha. In: Zierhut M, Ohno S, Orefice F, Pavesio CE, Rao NA. eds. Intraocular Inflammation. Berlin: Springer; 2016: 345-353
    CrossrefGoogle Scholar
  • 35 Shi J, Zhao C, Zhou J. et al. Effectiveness and safety of interferon α2a as an add-on treatment for refractory Behçetʼs uveitis. Ther Adv Chronic Dis 2019; 10: 1-9
    PubMedGoogle Scholar
  • 36 Bettiol A, Silvestri E, Di Scala G. et al. The right place of interleukin-1 inhibitors in the treatment of Behçetʼs syndrome: a systematic review. Rheumatol Int 2019; 39: 971-990
    CrossrefPubMedGoogle Scholar
  • 37 Yazici Y. Management of Behçet syndrome. Curr Opin Rheumatol 2020; 32: 35-40
    CrossrefPubMedGoogle Scholar
  • 38 Mamo JG. The rate of visual loss in Behçetʼs disease. Arch Ophthalmol 1970; 84: 451-452
    CrossrefPubMedGoogle Scholar
  • 39 Benezra D, Cohen E. Treatment and visual prognosis in Behçetʼs disease. Br J Ophthalmol 1986; 70: 589-592
    CrossrefPubMedGoogle Scholar
  • 40 Celiker H, Kazakoglu H, Direskeneli H. Conventional immunosuppressive therapy in severe Behçetʼs uveitis: the switch rate to the biological agents. BMC Ophthalmol 2018; 18: 261
    CrossrefPubMedGoogle Scholar