Aktuelle Neurologie 2010; 37(10): 505-517
DOI: 10.1055/s-0030-1265986
Übersicht
© Georg Thieme Verlag KG Stuttgart · New York

Immuntherapie bei Myasthenia gravis und Lambert-Eaton-Syndrom

Teil 1: Medikamentöse ImmunsuppressionImmunotherapy for Myasthenia Gravis and Lambert-Eaton Myasthenic SyndromePart 1: Drug-Induced ImmunosuppressionT.  Henze1 , R.  W. C.  Janzen2 , F.  Schumm3 , A. Melms4 , J.  P.  Sieb5 , W.  Köhler6 , F.  Heidenreich7 , B.  Tackenberg8 für den Ärztlichen Beirat der Deutschen Myasthenie-Gesellschaft (DMG) C.  Weber-Schoendorfer9
  • 1Reha-Zentrum Nittenau, Nittenau
  • 2Bad Homburg
  • 3Neurologische Klinik Christophsbad, Göppingen
  • 4Neurologische Universitätsklinik Tübingen
  • 5Neurologische Klinik, Klinikum Stralsund
  • 6Fachkrankenhaus Hubertusburg, Neurologische Klinik, Wermsdorf
  • 7Heidenreich, Neurologische Klinik, Henriettenstift Hannover
  • 8Neurologische Universitätsklinik Marburg
  • 9Pharmakovigilanz- und Beratungszentrum für Embryonaltoxikologie, Berlin
Further Information

Publication History

Publication Date:
05 January 2011 (online)

Zusammenfassung

Die Behandlung der Myasthenia gravis (MG) und des Lambert-Eaton-Syndroms (LEMS) entsprechend den Leitlinien der DGN ist in der Regel sehr wirksam. Aufgrund des sehr individuellen Verlaufs beider Erkrankungen, medikamentöser Unverträglichkeiten oder schwerwiegender Begleiterkrankungen ist es allerdings nicht selten erforderlich, von den „klassischen” Immuntherapeutika (Kortikosteroide, Azathioprin) abzuweichen und andere Medikamente im Off-label-use einzusetzen, für die zwar zumeist eine schwächere Studienlage besteht, aber doch eine tragfähige klinische Evidenz gegeben ist. Der Ärztliche Beirat der Deutschen Myasthenie-Gesellschaft hat daher die verfügbaren wissenschaftlichen Daten zu diesen „Reservemedikamenten” (Methotrexat, Cyclophosphamid, Cyclosporin A, Mycophenolat Mofetil, Tacrolimus, Rituximab) hinsichtlich Indikationen und Zulassung, Studienergebnissen, praktischer Anwendung einschließlich Therapiemonitoring, unerwünschter Wirkungen und Komplikationsmöglichkeiten aktualisiert. Für jedes Medikament werden darüber hinaus Erfahrungswissen und Hinweise zur Anwendung in Schwangerschaft und Stillzeit gegeben. Diese Synopsis will therapeutische Möglichkeiten aufzeigen und Hinweise liefern, wie auch bei einem schweren Verlauf von MG oder LEMS Nutzen-Risiko-Abwägungen vorgenommen werden können. Sie können den zielgerechten Umgang mit dem unvermeidlichen Off-label-use der genannten Substanzen fördern. In einem 2. Teil wird dann auf die Therapie mit intravenösen Immunglobulinen, Plasmaaustausch und Immunadsorption eingegangen.

Abstract

The treatment of myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) is – according to the guidelines of the German Neurological Society – very effective in most patients. Nevertheless due to the individual course of both diseases, adverse drug effects or severe comorbidities, it is often necessary to stop the treatment with the „classical” immunotherapeutics such as corticosteroids and azathioprine and to change to other drugs in spite of the fact that experience using them is much more scarce and that the drugs can only be given off-label. The Medical Advisory Board of the German Myasthenia Society (DMG) has therefore updated the available scientific data concerning these drugs (methotrexate, cyclophosphamide, cyclosporine, mycophenolate mofetil, tacrolimus, rituximab) with respect to indications and approval, study results, practical application and monitoring of therapy as well as adverse events and complications. Moreover, for each of these drugs, some practical information concerning pregnancy and breast-feeding is given. This publication is intended to demonstrate additional treatment modalities even in severe cases of MG or LEMS. Moreover, it may offer some arguments for the off-label-use of these drugs. In a second part of this publication, the treatment with high-dose immunoglobulins, plasma exchange and immunoadsorption will be discussed.

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Prof. Dr. med. Thomas Henze

Reha-Zentrum Nittenau

Eichendorffstr. 21

93149 Nittenau

Email: t.henze@rz-ni.de

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