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Zeitschrift für Rheumatologie

, Volume 78, Issue 4, pp 333–338 | Cite as

Aktuelle Therapie der eosinophilen Granulomatose mit Polyangiitis (Churg-Strauss-Syndrom)

  • F. MoosigEmail author
  • J. Holle
Leitthema

Zusammenfassung

Zur Behandlung der eosinophilen Granulomatose mit Polyangiitis (EGPA) steht eine sehr viel geringere Datenlage zur Verfügung als bei den anderen ANCA(antinukleäre zytoplasmatische Antikörper)-assoziierten Vaskulitiden. Zugleich unterscheidet sich die EGPA auch am stärksten von diesen. Zur Orientierung dienen die deutschen und internationalen Empfehlungen. In Abhängigkeit von Aktivitätsbestimmung, Krankheitsausbreitung und Prognoseabschätzung erfolgt eine angepasste remissionsinduzierende Therapie. Bei günstigen Prognosefaktoren kann eine alleinige Glukokortikoidtherapie, zur Steroideinsparung evtl. ergänzt um ein mittelpotentes Immunsuppressivum, erfolgen. Bei ungünstiger Prognose bzw. gravierender Organbeteiligung ist zusätzlich eine hochpotente Immunsuppression, zumeist mit Cyclophosphamid, erforderlich. Bei Remission sollte eine Erhaltungstherapie in Analogie zu den anderen ANCA-assoziierten Vaskulitiden (AAV) erfolgen. Seit Neuestem steht mit Mepolizumab, einem IL-5-Antikörper, auch ein Biologikum zur Verfügung, dessen Stellenwert aber noch bestimmt werden muss.

Schlüsselwörter

Mepolizumab Vaskulitis Prognose Immunsuppression Biologikum 

Current treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Abstract

For the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) much less data are available when compared to the other anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). At the same time EGPA also differs in many aspects from AAVs. Treatment is guided by the German and international guidelines. An adapted induction therapy is chosen depending on the disease activity, manifestations and factors determining the prognosis. For patients without negative prognostic factors glucocorticoids alone may be sufficient. A medium potent immunosuppressive agent may be added in order to economize on steroids. For patients with severe organ manifestations and adverse prognostic factors, a highly potent immunosuppression usually with cyclophosphamide, is necessary. In cases of remission a maintenance therapy is recommended in the same way as for other AAVs. Recently, a biological, the IL-5 antibody mepolizumab has also become available, although its precise role still has to be established.

Keywords

Mepolizumab Vasculitis Prognosis Immunosuppression Biologicals 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

F. Moosig und J. Holle geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2018

Authors and Affiliations

  1. 1.Rheumazentrum Schleswig-Holstein MitteNeumünsterDeutschland

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