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Eosinophile Granulomatose mit Polyangiitis

Update zu Klassifikation und Management

Eosinophilic granulomatosis with polyangiitis

Update on classification and management

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Zusammenfassung

Die eosinophile Granulomatose mit Polyangiitis (EGPA) ist eine seltene chronisch entzündliche Systemerkrankung, die bei Patienten mit einem Asthma bronchiale auftritt und mit einer signifikanten Blut- und Gewebeeosinophilie einhergeht. Ein weiteres Charakteristikum ist eine Vaskulitis kleiner und/oder mittelgroßer Gefäße, die in Prodromalstadien der Erkrankung fehlen kann und daher heute nicht mehr obligater Teil der Krankheitsdefinition ist. Bei etwa einem Drittel der Patienten können Antikörper gegen neutrophile zytoplasmatische Antigene (ANCA) nachgewiesen werden. Die ANCA-positive und die ANCA-negative EGPA sind genetisch unterschiedliche Erkrankungen mit gemeinsamen klinischen Manifestationen, die aber in unterschiedlicher Häufigkeit auftreten. Eine kardiale Beteiligung ist mit einer schlechten Prognose assoziiert. Es kommt häufig zu permanenten Organschäden als Folge der Grunderkrankung oder der Therapie, insbesondere mit Glukokortikoiden (GC). Die Standardtherapie der EGPA besteht aus GC in Kombination mit Cyclophosphamid bei gravierendem Organbefall oder mittelpotenten Immunsuppressiva bei prognostisch günstigeren Manifestationen. Biologika halten zunehmend Einzug in die Therapie der EGPA. Der IL(Interleukin)-5-Antagonist Mepolizumab reduziert bei Patienten mit rezidivierender EGPA ohne schwere Organbeteiligungen das Rezidivrisiko und senkt den GC-Bedarf. In Analogie zum Vorgehen bei den anderen ANCA-assoziierten Vaskulitiden stellt der Einsatz von Rituximab bei ANCA-positiven EGPA-Patienten mit schwerem Vaskulitisrezidiv evtl. eine Option dar, auch wenn die formale Evidenz für ein solches Vorgehen derzeit noch gering ist und eine formale Zulassung fehlt.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare chronic inflammatory systemic disease that occurs in patients with bronchial asthma and is associated with significant blood and tissue eosinophilia. Another characteristic is vasculitis of small and/or medium-sized vessels, which may be absent in prodromal stages of the disease and is therefore no longer an obligatory part of the disease definition. Antineutrophil cytoplasmic antibodies (ANCA) can be detected in approximately one third of patients. The ANCA-positive and ANCA-negative EGPA are genetically distinct diseases with common clinical manifestations, which, however, occur with different frequencies. Cardiac involvement is associated with a poor prognosis. Permanent organ damage often occurs as a result of the underlying disease or treatment, especially with glucocorticoids (GC). The standard treatment of EGPA consists of GC in combination with cyclophosphamide for severe organ involvement or medium potency immunosuppressants for more prognostically favorable manifestations. Biologics are increasingly being used in the treatment of EGPA. The interleukin (IL) 5 antagonist mepolizumab reduces the risk of relapses and decreases the demand for GC in patients with relapsing EGPA without severe organ involvement. In analogy to the approach to other ANCA-associated vasculitides, the use of rituximab in ANCA-positive EGPA patients with severe vasculitis recurrence is a possible option, even though formal evidence for such an approach is currently low and formal approval is lacking.

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  1. Vaskulitiszentrum Süd, Klinik für Innere Medizin, Rheumatologie und Immunologie, Medius Kliniken – Akademisches Lehrkrankenhaus der Universität Tübingen, Eugenstr. 3, 73230, Kirchheim unter Teck, Deutschland

    Bernhard Hellmich

  2. Rheumazentrum Schleswig-Holstein Mitte, Neumünster, Deutschland

    Julia Holle & Frank Moosig

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B. Hellmich: Vortragshonorare: Boehringer, GSK, Roche, Vifor. Honorare für Beratungsleistungen: InflaRx, GSK, Roche, Vifor. J. Holle und F. Moosig geben an, dass kein Interessenkonflikt besteht.

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Bernhard Hellmich, Kirchheim unter Teck

Julia Holle, Neumünster

Frank Moosig, Neumünster

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Hellmich, B., Holle, J. & Moosig, F. Eosinophile Granulomatose mit Polyangiitis. Z Rheumatol 81, 286–299 (2022). https://doi.org/10.1007/s00393-021-01153-6

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