275th ENMC international workshop: Seronegative myasthenia gravis: An update paradigm for diagnosis and management, 9-11 February 2024, Hoofddorp, the Netherlands
- PMID: 39427494
- DOI: 10.1016/j.nmd.2024.104468
275th ENMC international workshop: Seronegative myasthenia gravis: An update paradigm for diagnosis and management, 9-11 February 2024, Hoofddorp, the Netherlands
Abstract
The 275th ENMC workshop on the diagnosis and management of seronegative myasthenia gravis (SNMG) was held on February 9-11, 2024. The participants included experts in the field of adult and pediatric MG together with patient representatives. This workshop aimed to redefine SNMG in view of recent diagnostic and therapeutic updates and to identify patient unmet needs. The workshop has highlighted considerable challenges in the SNMG diagnostic work-up. To date, SNMG confirmation is often controversial, given the absence of specific diagnostic tests; no recommendations from international panels of experts are available in literature; myopathies, congenital myasthenic syndromes and functional disorders are the commonest misdiagnoses. Improving the disease diagnosis is crucial to avoid long delays in receiving appropriate treatment. To this purpose, a comprehensive diagnostic algorithm achieved consensus. Moreover, a remarkable variability in SNMG response to therapy and long-term prognosis has also been highlighted.
Keywords: Acetylcholine receptor antibodies; Cell-based-assay; Low-density lipoprotein receptor-related protein 4 antibodies; Muscle-specific kinase antibodies; Myasthenia gravis; Radioimunoprecipitation assay; Seronegative myasthenia gravis.
Copyright © 2024.
Conflict of interest statement
Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: AE received honoraria for serving as SPIN Award jury member from Grifols, as speaker from UCB and Argenx, for participating in meetings and advisory boards from UCB and Alexion. She declares no competing interest regarding this manuscript. JP has received support for scientific meetings and honorariums for advisory work from Merck Serono, Chugai, Alexion, Roche, Amgen, UCB, Amplo. Grants from Alexion, Argenx, Amplo biotechnology. Shares in AstraZenica. She acknowledges partial funding to the trust by Highly specialised services NHS England for the CMS service. She is on the UK NHSE IVIG Committee and until April 2024 was on the ABN advisory group for neuromuscular diseases. JJGMV has been involved MG research sponsored by the Princes Beatrix Fonds, Health Holland and consultancies for Argen-X, Alexion, and NMD Pharma. Reimbursements were received by the LUMC. He is coinventor on patent applications based on MuSK-related research. The LUMC receives royalties for MuSK antibody assays. He is a member of the European Reference Network for Rare Neuromuscular Diseases [ERN EURO-NMD]. LM has received honoraria for speaking, advisory boards and compensation for congress participations from Sanofi Genzyme, Roche, Biogen, Amicus Therapeutics, Alexion, Argenx, UCB, Janssen, Lupin, outside the submitted work. US received honoraria for aboard attendance and invited talks from Alexion. DS has no relevant Conflicts of Interest to declare. NEG has received honoraria for advisory work and as a speaker from Aera, Alexion, Amgen, Argenx, Denka, Dianthus, Grifols, Huma, Immunovant, Johnson & Johnson (Janssen), Merck, Roche, Takeda, UCB. ECV has received public speaking honoraria and compensation for advisory boards and/or consultations fees from UCB, argenx, Alexion, Janssen and Lundbeck. She declares no competing interest regarding this manuscript. JH has received honoraria for serving as speaker for Roche and for participating in meetings and advisory boards for Merck and Argenx. She declares no competing interest regarding this manuscript. MBU MU has received honoraria for consulting services from Alexion, Janssen, Merck, and UCB, and speaking fees from Alexion and UCB. She also provided consulting services to Argenx. Travel expenses for Congress were paid by Alexion, UCB, and Argenx. MT reports consultancies for ArgenX, UCB Pharma, Johnson and Johnson, Peervoice and Medtalks, and research funding from ZonMW, NWO, ArgenX and NMD Pharma. All reimbursements were received by the Leiden University Medical Center. EN reports consultancies for Momenta Pharmaceuticals. He is also principal investigator at the LUMC for clinical trials in juvenile myasthenia gravis sponsored by ArgenX, Johnson and Johnson, and Alexion. All reimbursements were received by the Leiden University Medical Center. MC has received travelling grants for congress participations from Argenx, Roche and UCB. (Non son certa serva metterlo, ma in caso le mie disclosure). GS has received consultancies for Medthority - EPG Health (an IQVIA business) and is supported by the European Academy of Neurology Research Training Fellowship. He reports no competing interests regarding this manuscript. JV has received consultancy and/or speaker fees from ArgenX, UCB Pharma, Toleranzia, Novartis, Johnson and Johnson (Janssen), Merck, Alexion, Horizon Therapeutics (Amgen) and Dianthus Therapeutics. He declares no competing interests regarding this manuscript. ARP has obtained consultancy fees from ArgenX, UCB Pharma, Alexion, Toleranzia, Novartis and Dianthus Therapeutics. VD has received public speaking honoraria and compensation for advisory boards and/or consultations fees from UCB, Alexion, Dianthus, Roche. She declares no competing interest regarding this manuscript. SR has received support for scientific meetings, speaker fees and honorarium for advisory work from Novartis, Sarepta, Argenx, UCB and Roche.
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