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Miastenia grave

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Last reviewed: 24 Sep 2025
Last updated: 14 Aug 2025
14 Aug 2025

Nipocalimab aprobado por la FDA para el tratamiento de la miastenia grave generalizada

Nipocalimab, un anticuerpo monoclonal antagonista neonatal del receptor Fc (FcRn), ha sido aprobado por la Administración de Medicamentos y Alimentos de EE. UU. (FDA) para el tratamiento de la miastenia grave generalizada en adultos y niños ≥12 años que son positivos para anticuerpos contra el receptor de acetilcolina (AChR) o la tirosina quinasa específica del músculo (MuSK). La aprobación sigue a la designación de revisión prioritaria de la FDA y proporciona una opción de tratamiento alternativa para ofrecer un control sostenido de la enfermedad para una amplia gama de pacientes con síntomas molestos causados por la miastenia grave.

La aprobación se basó en los resultados de Vivacity-MG3, un ensayo de fase 3 de pacientes con miastenia grave generalizada inadecuadamente controlada con la terapia estándar. El nipocalimab agregado a la atención estándar se asoció con reducciones duraderas en los anticuerpos IgG y AChR circulantes totales, y mejoras en los resultados informados por los pacientes, con un buen perfil de seguridad.

See Management: approach

Original source of update

Summary

Definition

History and exam

Key diagnostic factors

  • fuerza muscular fatiga
  • ptosis
  • diplopía
  • disfagia
  • disartría
  • paresia facial
  • debilidad en las extremidades proximales
  • disnea
Full details

Risk factors

  • antecedentes familiares de trastornos autoinmunes
  • marcadores genéticos
  • tratamiento dirigido contra el cáncer
Full details

Diagnostic tests

1st tests to order

  • análisis sérico de anticuerpos frente a los receptores de acetilcolina (AChR)
  • anticuerpos frente a la tirosina cinasa específica del músculo (MuSK)
  • pruebas funcionales respiratorias en serie
Full details

Tests to consider

  • ensayos de anticuerpos contra el receptor estriado
  • estimulación nerviosa repetitiva
  • electromiograma (EMG) de fibra aislada
  • tomografía computarizada (TC) de tórax
Full details

Treatment algorithm

ACUTE

crisis miasténica

ONGOING

enfermedad de leve a moderada (clase I a III)

enfermedad grave (clase IV o V) o refractaria

Contributors

Authors

David P. Richman, MD

Distinguished Professor

Professor of Neurology

University of California - Davis

Davis

CA

Disclosures

DPR declares that he has no competing interests.

Acknowledgements

Dr David Richman would like to gratefully acknowledge Dr Robert Lisak, Dr Andrea Corse, and Dr Ami Mankodi, previous contributors to this topic.

Disclosures

AC and AM declare that they have no competing interests. RPL is a Data and Safety Monitoring Board Member for the COUR myasthenia gravis clinical trial. RPL is a site principal investigator for clinical trials and a co-author for the clinical trial reports for myasthenia gravis therapies for Alexion, Argenx, and UCB Ra. RPL's institution receives payment for the time spent on these clinical trials. RPL has received book royalties from Oxford University Press and Blackstone, and has carried out consultancy work for Avilar.

Peer reviewers

Vern C. Juel, MD

Associate Professor of Medicine (Neurology)

Duke University

Durham

NC

Disclosures

VCJ declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Narayanaswami P, Sanders DB, Wolfe G, et al. International consensus guidance for management of myasthenia gravis: 2020 update. Neurology. 2021 Jan 19;96(3):114-22.Full text  Abstract

Skeie GO, Apostolski S, Evoli A, et al. Guidelines for treatment of autoimmune neuromuscular transmission disorders. Eur J Neurol. 2010 Jul;17(7):893-902.Full text  Abstract

Gronseth GS, Barohn R, Narayanaswami P. Practice advisory: thymectomy for myasthenia gravis (practice parameter update). Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2020 Apr 21;94(16):705-9.Full text  Abstract

Elovaara I, Apostolski S, van Doorn P, et al. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases. Eur J Neurol. 2008 Sep;15(9):893-908.Full text  Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
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