Morbus Behçet- eine autoinflammatorische Erkrankung?

Zusammenfassung

In letzter Zeit wird die Zugehörigkeit des Morbus Behçet zum Formenkreis der autoinflammatorischen Erkrankungen diskutiert. In der vorliegenden Arbeit werden die Gründe hierfür zusammengefasst. Es handelt sich vor allem vor allem um die Ähnlichkeit der Symptomatologie des M. Behçet mit derjenigen verschiedener autoinflammatorischer Erkrankungen, aber auch immunologische Befunde deuten zumindest auf ähnliche pathogenetische Mechanismen hin – so zum Beispiel die wesentliche Beteiligung des angeborenen („innate”) Immunsystems, die Hyperreagibilität von Zellen dieses Immunsystems auf zum Beispiel mikrobielle Reize („Stress”). Auch wenn bislang keine der bei klassischen autoinflammatorischen Erkrankungen bekannten genetischen Mutationen gefunden werden konnten, so gibt es doch auch molekulargenetisch indirekte Hinweise wie zum Beispiel die überzufällige Häufung von Mutationen im MEFV Gen (familiäres Mittelmeerfieber, FMF) bei Patienten mit M. Behçet in Ländern, in denen beide Erkrankungen endemisch vorkommen. Des Weiteren wirken ähnliche therapeutische Prinzipien bei M. Behçet und bei autoinflammatorischen Erkrankungen. Colchicin ist nicht nur bei M. Behçet, sondern auch bei FMF und bei der Gicht wirksam, Interferon-alpha wirkt bei M. Behçet und therapierefraktärem FMF und IL-1 Antagonisten scheinen ebenfalls nicht nur bei monogenen autoinflammatorischen Erkrankungen, sondern auch bei FMF, Gicht und M.Behçet effektiv zu sein.

Abstract

During the last years, it is being increasingly discussed whether Behçet's disease (BD) belongs to the group of autoinflammatory diseases. The present article summarises the reasons for this hypothesis. Mainly, this is based on the similarities in symptomatology, but also on immunological findings such as the major impact that the innate immune system has on the pathogenesis of BD, the hyperreactivity of cells of the innate immune system in BD reacting inadequately on microbial or other signals (“stress”). Although to date none of the known genetic mutations has been found in BD, there are some indirect hints on molecular genetic aberrations, such as the dysproportionally high frequency of MEFV mutations (familial Mediterranean fever, FMF) in patients with BD in countries where both diseases are endemic. Moreover, similar therapeutic principles are effective in BD and autoinflammatory diseases. Colchicine is effective in BD, but also in gout and FMF, interferon-alpha is effective in BD and in treatment-resistant FMF, and IL-1 antagonists not only are effective in monogenic autoinflammatory diseases, but also in FMF, gout and BD.

Schlüsselwörter

Morbus Behçet - autoinflammatorische Erkrankungen - Immunologie - Therapie

Key words

Behçet's disease - autoinflammatory disease - immunology - treatment

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