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Aktuelle Rheumatologie 2019; 44(04): 262-275
DOI: 10.1055/a-0979-4510
Übersichtsarbeit
© Georg Thieme Verlag KG Stuttgart · New York

Behçet-Syndrom

Behçet’s Syndrome
Theodoros Xenitidis
1   Abteilung und Lehrstuhl II, Hämatologie/Onkologie/klinische Immunologie/Rheumatologie, Medizinische Universitätsklinik, Universitätsklinikum Tübingen
,
Joerg Christoph Henes
1   Abteilung und Lehrstuhl II, Hämatologie/Onkologie/klinische Immunologie/Rheumatologie, Medizinische Universitätsklinik, Universitätsklinikum Tübingen
› Author Affiliations
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Publication History

Publication Date:
13 August 2019 (online)

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Zusammenfassung

Das Behçet-Syndrom gehört zu den Vasculitiden variabler Gefäßgröße. Das klinische Bild ist sehr vielfältig und erfordert in der Regel eine interdisziplinäre Zusammenarbeit. Pathogenetisch scheint es eine Mittelstellung zwischen einer polygenetischen Autoinflammationserkrankung und einer autoimmunen Erkrankung einzunehmen. 2018 wurden neue EULAR-Empfehlungen herausgegeben. Die Therapie richtet sich nach der führenden Beteiligung der einzelnen Organe. Seit 2016 ist Adalimumab zugelassen für die Behandlung der hinteren Augenbeteiligung. Infliximab, Interferon a2a, Interleukin-1-Antagonisten und Apremilast können Therapiealternativen darstellen.

Abstract

Behçet’s syndrome (BS) is classified as a variable vessel vasculitis. The clinical picture is very diverse and usually requires interdisciplinary collaboration. Pathogenetically, BS seems to take a middle position between a polygenic autoinflammatory disease and an autoimmune disease. New EULAR recommendations were issued in 2018. The therapy depends on which organs are most affected. Since 2016, adalimumab has been approved for the treatment of posterior ocular involvement. Infliximab, interferon a2a, interleukin-1 antagonists and apremilast may be alternative therapies.

Schlüsselwörter

Vaskulitis - Review - Therapie - Behçet-Syndrom

Key words

Vasculitis - Review - Therapy - Behçet’s syndrome
 

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