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

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

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