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Management der ANCA-assoziierten Vaskulitiden

Management of ANCA-associated vasculitides

  • Schwerpunkt: Vaskulitiden
  • Published:
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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.

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune-mediated inflammation of small and medium-sized vessels that can affect virtually any organ system and bears the risk of irreversible organ damage. Without treatment the mortality rates are high, which necessitates rapid diagnosis and initiation of treatment. Histological confirmation, which is not feasible in all cases, should be strived for, especially to delineate differential diagnoses and vasculitis mimics. The new American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria are primarily designed for study purposes and show limitations in the routine application. Globally, the recently updated EULAR recommendations represent the most up to date management guidelines. Therapeutically, rituximab and cyclophosphamide in combination with glucocorticoids remain the pillars of treatment in remission induction for severe organ-threatening and life-threatening diseases. For the first time, mepolizumab and avacopan represent approved treatment options for specific entities that make a significant contribution to steroid reduction. New attention has been paid to patient-reported outcomes, for which a disease-specific outcome questionnaire is now available.

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  1. Klinik für Innere Medizin, Rheumatologie, Pneumologie, Nephrologie und Diabetologie, medius Klinik Kirchheim, Eugenstr. 3, 73230, Kirchheim unter Teck, Deutschland

    Christian Löffler & Bernhard Hellmich

  2. Vaskulitis-Referenzzentrum der Europäischen Union ERN-RITA, Lehrkrankenhaus der Eberhard Karls Universität Tübingen, Kirchheim unter Teck, Deutschland

    Christian Löffler & Bernhard Hellmich

  3. Klinik für Nephrologie, Endokrinologie, Hypertensiologie und Rheumatologie, Universitätsmedizin Mannheim, Medizinische Fakultät der Universität Heidelberg, Mannheim, Deutschland

    Christian Löffler

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Tab. S1: Dosierungen für Induktionstherapie bei Granulomatose mit Polyangiitis (GPA)/mikroskopischer Polyangiitis (MPA); Tab. S2: Dosierungen für Erhaltungstherapie bei Granulomatose mit Polyangiitis (GPA)/mikroskopischer Polyangiitis (MPA); Tab. S3: Dosierungen für Induktionstherapie bei eosinophiler Granulomatose mit Polyangiitis (EGPA); Tab. S4: Dosierungen für Erhaltungstherapie bei eosinophiler Granulomatose mit Polyangiitis (EGPA)

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Löffler, C., Hellmich, B. Management der ANCA-assoziierten Vaskulitiden. Innere Medizin 65, 93–106 (2024). https://doi.org/10.1007/s00108-023-01655-2

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  • DOI: https://doi.org/10.1007/s00108-023-01655-2

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