Notfallsituationen bei Morbus Behçet

Zusammenfassung

Als internistische, zum Teil auch chirurgische oder neurologische Notfallsituation beim M. Behçet stellen sich vor allem arterielle und hier insbesondere pulmonalarterielle Aneurysmen dar, die aufgrund von Blutungen auch die häufigste Todesursache sind. Prognostisch ebenfalls ungünstig, und mit einer 10-Jahres Mortalität von 10% behaftet, sind die Manifestationen am ZNS, allen voran die sterile Meningoenzephalitis. Ebenfalls problematisch ist die gastrointestinale Beteiligung, die durch Perforation von Ulcera zum Tode führen kann. Venöse Thrombosen sind vor allem dann problematisch, wenn die Vena cava superior betroffen ist. Letztlich muss auch die okuläre Manifestation (retinale Vasklitis) als Notfall betrachtet werden, da sie unbehandelt in ca. 70% der Fälle zur Erblindung führt. Die therapeutischen Möglichkeiten für die schweren Manifestationen des M. Behçet haben sich in den letzten 10 Jahren, vor allem durch die TNF Blocker und alpha-Interferon, deutlich verbessert.

Abstract

Emergency situations in Behçet's disease for the internist, surgeon or neurologist consist of arterial and mainly pulmonary arterial aneurysms which are the main cause of death by bleedings. Neurological manifestations in the CNS such as meningoencephalitis have a bad prognosis with a 10% 10-year mortality. Gastrointestinal manifestations may lead to death by perforation of ulcerations. Venous thrombembolism is dangerous, when the inferior vena cava is affected. Finally, also ocular manifestations (retinal vasculitis) should be considered as emergency situations, as without treatment, they lead to blindness in 70% of cases. The therapeutic possibilities have greatly improved in the last 10 years, mainly by the introduction of anti-TNF-agents and interferon-alpha in the management of Behçet's disease.

Schlüsselwörter

Morbus Behçet - Notfall - arterielle Aneurysmen - ZNS Manifestationen

Key words

Behçet's disease - emergency - arterial aneurysms - CNS manifestations

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