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Morbus Behçet

Behçet’s disease

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Zusammenfassung

Der Morbus Behçet ist eine Systemerkrankung mit dem histologischen Korrelat einer leukozytoklastischen Vaskulitis. Pathogenetisch werden neben einer starken genetischen Komponente eine Beteiligung des angeborenen Immunsystems sowie auch eine autoinflammatorische Komponente diskutiert. Die Erkrankung ist am häufigsten in Ländern entlang der früheren Seidenstraße, in Deutschland ist die Erkrankung dagegen selten. Hauptsymptome sind orale Aphthen und Hautmanifestationen, gefolgt von genitalen Aphthen und einer Oligoarthritis der großen Gelenke. Schwerwiegende Manifestationen sind die gastrointestinale Manifestation mit Perforationsneigung, die arteriellen, vor allem pulmonalarteriellen Aeurysmen, die ZNS-Manifestationen und die Augenbeteiligung. Therapeutisch kommen bei leichten Krankheitsmanifestationen niedrig dosierte Steroide und Colchicin zum Einsatz, bei mittelgradigen Manifestationen Azathioprin und Steroide, alternativ Cyclosporin A und Steroide, bei schweren Manifestationen Interferon-α, TNF-Antagonisten oder Zytostatika. In Studien werden aktuell Interleukin-1-Antagonisten erprobt.

Abstract

Behçet’s disease is a systemic disorder with the histopathological correlate of leukocytoclastic vasculitis. Pathogenetically, besides a strong genetic component participation of the innate immune system and an autoinflammatory component are discussed. The disease is most common in countries along the former silk route but in Germany the disease is rare (prevalence approximately 0.6/100,000). Oral aphthous ulcers are the main symptom, followed by skin manifestations, genital ulcers and oligoarthritis of large joints. Severe manifestations, threatening quality of life and even life itself, are the gastrointestinal manifestations which often perforate, arterial, mainly pulmonary arterial aneurysms which cause life-threatening bleeding, CNS manifestations and ocular disease, which with occlusive retinal vasculitis often leads to blindness. For milder manifestations low-dose steroids and colchicine are used, for moderate manifestations such as arthritis or ocular disease not immediately threatening visual acuity, azathioprin or cyclosporin A are combined with steroids. For severe manifestations, interferon-alpha, TNF-antagonists or cytotoxic drugs are recommended. Interleukin 1 (IL-1) antagonists are currently being examined in clinical studies.

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Kötter, I., Xenitidis, T., Fierlbeck, G. et al. Morbus Behçet. Z. Rheumatol. 71, 685–697 (2012). https://doi.org/10.1007/s00393-012-1012-x

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