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Nervenheilkunde 2014; 33(07/08): 519-525
DOI: 10.1055/s-0038-1627706
Fortbildung Karlsruhe
Schattauer GmbH

Myasthenia gravis: Was gibt es Neues?

What’s new in myasthenia gravis?
J. P. Sieb
1   Neurologische Klinik, HELIOS Hanseklinikum Stralsund
› Author Affiliations
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Publication History

eingegangen am: 26 November 2013

angenommen am: 28 February 2014

Publication Date:
24 January 2018 (online)

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Zusammenfassung

Der Begriff Myasthenia gravis umfasst eine heterogene Gruppe von Autoimmunerkrankungen mit einem postsynaptischen Defekt der neuromuskulären Signalübertragung. Eine Unterteilung ist möglich nach Antikörperspezifität (Acetylcholinrezeptor, MuSK, LRP4, seronegativ), Thymushistologie (Thymitis, Thymom, normal / Atrophie), Erkrankungsbeginn (vor oder nach dem 50. Lebensjahr) und Verlaufstyp (okulär, oropharyngeal, generalisiert). Die etablierten Therapieoptionen stehen seit Jahrzehnten zur Verfügung. Dies sind die Acetylcholinesteraseinhibition, die Immunsuppression mit einem Glukokortikosteroid und in der Dauertherapie Azathioprin, die Thymektomie und Krisenintervention mit einem Aphereseverfahren bzw. i.v.-Immunglobulinen. Möglicherweise wird sich mit der Einführung von monoklonalen Antikörpern das therapeutische Vorgehen maßgeblich ändern. Bereits heute sollte Rituximab bei generalisierten, therapierefraktären Krankheitsverläufen erwogen werden. Aussichtsreiche Kandidaten sind Belimumab, der die Ausdifferenzierung von B-Zellen hemmt, und Eculizumab, der an das Komplementprotein C5 bindet und die Komplementaktivierung hemmt.

Summary

This is an update on the diagnosis and treatment of myasthenia gravis. Today the term myasthenia gravis comprises a heterogeneous group of autoimmune diseases with a postsynaptic defect of neuromuscular transmission. These diseases can be classified according to antibody specificity (acetylcholine receptor, MuSK, LRP4, seronegative), thymus histology (thymitis, thymoma, normal/ atrophic), onset (before or after age 50) and course type (ocular, oropharyngeal, generalized). The established treatment options were already introduced decades ago. These are the acetylcholinesterase inhibition, prednisolone and azathioprine for the long-term immunosuppression, thymectomy and crisis intervention with an apheresis procedure or intravenous immunoglobulins. However, the introduction of monoclonal antibodies could substantially change the therapeutic approach in the forthcoming decade. Rituximab should already be considered in cases refractory to conventional treatment nowadays. Promising candidates are belimumab that inhibits the differentiation of B cells, and eculizumab, which binds to the complement protein C5 and hereby inhibits complement activation.

Schlüsselwörter

Acetylcholinrezeptor - Immunsuppression - Muskelschwäche - Thymektomie

Keywords

Acetylcholine receptor - immunosuppression - muscular weakness - thymectomy
 

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