Morbus Behçet: Eine interdisziplinäre Herausforderung aus ophthalmologischer Sicht

Zusammenfassung

Der Morbus (M.) Behçet (auch Behçet Erkrankung oder Adamantiades-Behçet-Syndrom) ist ein systemisches entzündliches Krankheitsbild unbekannter Ursache. In der aktuellen Chapel-Hill-Nomenklatur für die Vaskulitiden wird der M. Behçet als Vaskulitis variabler Gefäße klassifiziert, da Arterien und Venen aller Kaliber in unterschiedlichem Ausmaß betroffen sind. Sowohl das angeborene als auch das adaptive Immunsystem sind bei der Pathogenese der Erkrankung beteiligt. Es liegen überwiegend Merkmale einer autoinflammatorischen Erkrankung vor, weswegen aktuell diskutiert wird, dass es sich bei der Erkrankung um ein Mischbild zwischen Autoimmunität und Autoinflammation handelt. Die Krankheit ist durch Exazerbationen und schubartigen Verlauf unterschiedlicher Symptome und Organmanifestationen gekennzeichnet. Sie kann durch geringe Haut- und Schleimhautläsionen in Erscheinung treten oder zu schwerwiegenden Augenbeteiligungen mit Erblindungsfolge führen. Zusätzliche Beteiligungen des Magen-Darm-Traktes, des Nervensystems und der großen Gefäße sind manchmal lebensbedrohlich. Die Behandlung des M. Behçet umfasst ein breites Spektrum von immunmodulatorischen Wirkstoffen mit zunehmender Bedeutung von Biologika. So konnte die IFN-α-Therapie für die intraokulare Entzündung eine signifikante Wirksamkeit belegen. Monoklonale Antikörper gegen Tumornekrosefaktor (TNF)-α und Interleukin-1 wurden in klinischen Studien erfolgreich geprüft und erstere inzwischen in einigen Ländern zugelassen. In dieser Übersichtsdarstellung stellen wir den aktuellen Stand des Wissens zusammen und betonen insbesondere die Rolle des Augenarztes bei der Diagnostik und Behandlung des M. Behçet.

Abstract

Behçet’s disease (BD) (also known as Adamantiades-Behçet syndrome) is a systemic inflammatory disorder of unknown etiology. In the current Chapel Hill Nomenclature for vasculitides, it is classified as variable vessel vasculitis as it affects arterial and venous vessels of all sizes. Both the innate and the adaptive immune system are involved in the pathogenesis of BD. The disorder is largely characterised by the features of an autoinflammatory disease and is therefore currently considered to be at the crossroads between autoimmune and autoinflammatory syndromes. The disease typically involves exacerbations and remissions of symptoms and organ manifestations. It may produce only mild mucocutaneous lesions, but may also lead to severe ocular involvement resulting in blindness. Additional involvement of the gastrointestinal tract, CNS and large vessels may be life-threatening. The treatment of BD includes a broad spectrum of immunomodulatory agents with increasing importance of biologic agents. IFN-α therapy has shown significant efficacy for intraocular inflammation. Monoclonal antibodies against tumour necrosis factor (TNF)-α and interleukin-1 have been successful in clinical trials, and the former has been approved in several countries. This article summarises the current state of knowledge and emphasises the role of ophthalmologists in the management of BD.

Schlüsselwörter

Autoinflammation - Morbus Behçet - Biologika - Therapie - Uveitis

Key words

autoinflammation - Behçet’s disease - biologics - treatment - uveitis

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