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22.01.2024 | Churg-Strauss-Syndrom | Schwerpunkt: Vaskulitiden

Management der ANCA-assoziierten Vaskulitiden

verfasst von: PD Dr. Christian Löffler, Bernhard Hellmich

Erschienen in: Die Innere Medizin | Ausgabe 2/2024

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Zusammenfassung

Mit antineutrophilen zytoplasmatischen Antikörpern (ANCA) assoziierte Vaskulitiden (AAV) sind autoimmune Entzündungen kleiner und mittelgroßer Gefäße, die praktisch jedes Organsystem befallen können und das Risiko irreversibler Organschäden bergen. Unbehandelt besteht eine sehr hohe Mortalität, was eine rasche Diagnosestellung und Therapieeinleitung nötig macht. Eine histologische Sicherung, die nicht in allen Fällen möglich ist, sollte angestrebt werden, insbesondere um Differenzialdiagnosen und Vaskulitis-Mimics abzugrenzen. Die neuen Klassifikationskriterien des American College of Rheumatology (ACR) und der European Alliance of Associations for Rheumatology (EULAR) sind primär für Studienzwecke konzipiert und zeigen Limitationen in der Alltagsanwendung. Die kürzlich aktualisierten EULAR-Empfehlungen stellen global die aktuellsten Managementleitlinien dar. Therapeutisch bleiben Rituximab und Cyclophosphamid in Kombination mit Glukokortikoiden die Therapiesäulen für schwere, organ- und lebensbedrohende Erkrankungen in der Remissionsinduktion. Mit Mepolizumab und Avacopan stehen erstmals für spezifische Entitäten zugelassene Therapieoptionen zur Verfügung, die wesentlich zur Steroidreduktion beitragen. Neue Aufmerksamkeit haben die von Patienten berichteten Endpunkte erlangt, für die es bei den AAV nun erstmals einen krankheitsspezifischen Outcomefragebogen gibt.

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https://www.g-ba.de/downloads/40-268-8727/2022-08-04_AM-RL-XII_Avacopan_D-778_TrG.pdf

Titel: Management der ANCA-assoziierten Vaskulitiden
verfasst von: PD Dr. Christian Löffler
Bernhard Hellmich
Publikationsdatum: 22.01.2024
Verlag: Springer Medizin
Schlagwörter: Churg-Strauss-Syndrom
Churg-Strauss-Syndrom
Rituximab
Rituximab
Wegener-Granulomatose
Wegener-Granulomatose
Vaskulitiden
Vaskulitiden
Mepolizumab
Erschienen in: Die Innere Medizin / Ausgabe 2/2024
Print ISSN: 2731-7080
Elektronische ISSN: 2731-7099
DOI: https://doi.org/10.1007/s00108-023-01655-2

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