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Miastenia gravis

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Last reviewed: 16 Aug 2025
Last updated: 14 Aug 2025
14 Aug 2025

Nipocalimabe aprovado pela FDA para tratamento de miastenia gravis generalizada

O nipocalimabe, um anticorpo monoclonal antagonista dos receptores Fc neonatais (FcRn), foi aprovado pela Food and Drug Administration (FDA) dos EUA para o tratamento de miastenia gravis generalizada em adultos e crianças ≥12 anos que são positivos para anticorpos antirreceptor de acetilcolina (AChR) ou antitirosina quinase músculo específica (MuSK). A aprovação segue a designação de revisão prioritária da FDA e fornece uma opção de tratamento alternativa para oferecer controle sustentado da doença para uma ampla gama de pacientes com sintomas incômodos causados pela miastenia gravis.

A aprovação foi baseada nos resultados do Vivacity-MG3, um estudo de fase 3 com pacientes com miastenia gravis generalizada inadequadamente controlada com a terapia padrão. O nipocalimabe adicionado ao padrão de cuidados foi associado a reduções duradouras nos anticorpos IgG e AChR circulantes totais e melhoras nos desfechos relatados pelos pacientes, com um bom perfil de segurança.

See Management: approach

Original source of update

Summary

Definition

History and exam

Key diagnostic factors

  • fatigabilidade da força muscular
  • ptose
  • diplopia
  • disfagia
  • disartria
  • paresia facial
  • fraqueza muscular proximal
  • dispneia
Full details

Risk factors

  • história familiar de distúrbios autoimunes
  • marcadores genéticos
  • terapia dirigida ao câncer
Full details

Diagnostic investigations

1st investigations to order

  • análise de anticorpo sérico antirreceptor da acetilcolina (AchR)
  • anticorpos antitirosina quinase músculo-específica (MuSK)
  • testes seriados de função pulmonar
Full details

Investigations to consider

  • testes de anticorpos antirreceptor de músculo estriado
  • estimulação nervosa repetitiva
  • eletromiografia (EMG) de fibra única
  • tomografia computadorizada (TC) do tórax
Full details

Treatment algorithm

ACUTE

crise miastênica

ONGOING

doença leve a moderada (classe I a III)

doença grave (classe IV ou V) ou refratária

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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