Behçet- und Cogan-Syndrom

Zusammenfassung

Das Behçet- und das Cogan-Syndrom bilden zusammen die Gruppe der Vaskulitiden der variablen Gefäße nach der Chapel-Hill Nomenklatur. Sie können Arterien und Venen jeder Größe betreffen. Wie die Bezeichnung „Syndrom“ bei beiden Krankheitsbildern bereits widerspiegelt, können beide individuell sehr unterschiedliche Symptome bieten. Beide zählen formal zu den seltenen Erkrankungen, wobei das Cogan-Syndrom mit der Beschreibung von lediglich einigen hundert Fällen weltweit deutlich seltener als das Behςet-Syndrom ist. Für letzteres gibt es Diagnose- und Klassifikationskriterien, und auch europäische (EULAR, European Alliance of Associations for Rheumatology) Therapieempfehlungen. Symptomatologie, Diagnostik und Therapie, aber auch einige Überlegungen zur Pathogenese dieser beiden Vaskulitiden werden im Folgenden beleuchtet.

Abstract

Behçet’s syndrome and Cogan’s syndrome constitute the group of variable vessel vasculitides in the Chapel-Hill Nomenclature. They involve arteries and veins of all sizes. As reflected in the name “syndrome”, both diseases can manifest with different individual symptoms. Both formally are rare diseases, but the Cogan syndrome is much rarer than Behçet`s. For the latter, there are diagnosis and classification criteria as well as European (EULAR, European Alliance of Associations for Rheumatology) treatment recommendations. The symptomatology, diagnostic measures and treatment as well as some considerations about pathogenesis will be discussed in this article.

Schlüsselwörter

Behçet - Cogan - Pathogenese - Diagnose - Therapie - Behçet - Cogan - Pathogenesis - Diagnosis - Treatment

Publication History

Received: 07 August 2022

Accepted: 15 August 2022

Article published online:
21 November 2023

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