Journal Article Ppublish
PubMed: 42717567 DOI: 10.1111/ene.70747
Practical Guidance on Initiating and Switching Targeted Immunotherapies in Generalised Myasthenia Gravis: A German-Austrian Expert Opinion Paper.
Eur J Neurol 33(9):e70747
Authors: Andreas Meisel, Adela Della Marina, Paolo Doksani, Tim Hagenacker, Christoph Kleinschnitz, Heidrun H Krämer, Jan D Lünemann, Sven G Meuth, Tobias Ruck, Ulrike Schara-Schmidt, Christiane Schneider-Gold, Benedikt Schoser, Michael Schroeter, Charlotte Schubert, Jörn Peter Sieb, Fritz Zimprich, Jana Zschüntzsch, Sarah Hoffmann
Affiliations
- Department of Neurology With Experimental Neurology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
- Neuroscience Clinical Research Center, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
- Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
- Department of Pediatric Neurology, Center for Neuromuscular Disorders in Children and Adolescents, University Hospital Essen, University Duisburg-Essen, Essen, Germany.
- Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, Essen, Germany.
- Department of Neurology, Justus-Liebig-University Giessen, Giessen, Germany.
- Division of Neurology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada. Djavad Mowafaghian Centre for Brain Health, University of British Columbia, Vancouver, British Columbia, Canada. School of Biomedical Engineering, University of British Columbia, Vancouver, British Columbia, Canada. Department of Neurology, University Hospital Münster, Münster, Germany.
- Department of Neurology, Heinrich-Heine-University Düsseldorf, Düsseldorf, Germany.
- Department of Neurology, Ruhr University Bochum, BG University Hospital Bergmannsheil, Heimer Institute for Muscle Research, Bochum, Germany.
- Department of Neurology, St. Josef Hospital, Ruhr-University Bochum, Bochum, Germany.
- Friedrich-Baur-Institut Neurologische Klinik und Poliklinik LMU Klinikum München, Friedrich-Baur-Institute, Department of Neurology, LMU Clinics Munich, Munich, Germany.
- Department of Neurology, University of Cologne and University Hospital, Cologne, Germany.
- Department of Neurology, Institute of Neuroimmunology and MS (INIMS), University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
- Department of Neurology, Helios Hanseklinikum Stralsund, Stralsund, Germany.
- Department of Neurology, Medical University of Vienna, Vienna, Austria.
- Department of Neurology, University Medical Center Göttingen, Göttingen, Germany.
Abstract
BACKGROUND
The therapeutic landscape of generalised myasthenia gravis (gMG) has evolved substantially with the approval of targeted immunotherapies, including complement C5 inhibitors (C5-I) and neonatal Fc receptor inhibitors (FcRn-I). While pivotal trials have demonstrated marked efficacy in defined subgroups, real-world experience reveals more heterogeneous outcomes and raises questions about optimal patient selection, therapy sequencing and integration into clinical practice. In Germany and Austria, early access following regulatory approval has facilitated clinical experience over recent years. This expert opinion paper aims to combine current evidence with clinical experience to guide the use of C5-I and FcRn-I in everyday care.
METHODS
A panel of 18 neurologists from Germany and Austria, including two paediatric neurologists, evaluated study data and real-world experiences. Through structured discussion and a consensus process, they developed evidence- and experience-based recommendations on integrating targeted immunotherapies into existing treatment algorithms.
RESULTS
The panel provides practical recommendations for managing (highly) active gMG in adults, focusing on C5-I (eculizumab, ravulizumab, zilucoplan) and FcRn-I (efgartigimod, rozanolixizumab). The statements cover initiation criteria, sequencing and switching within and between drug classes and transitions to intensified immunomodulatory therapies (rituximab, apheresis, intravenous or subcutaneous immunoglobulin), as well as special considerations for juvenile MG.
CONCLUSIONS
This expert statement provides a practice-oriented framework integrating current evidence and clinical experience to support individualised therapeutic decision-making in gMG.
MeSH
Complement C5, Complement Inactivating Agents, Immunotherapy, Therapeutics, Nervous System Diseases, Neurologists, Myasthenia Gravis