Management of Myasthenia Gravis: International Consensus Guidance
Written By : Medha Baranwal
Medically Reviewed By : Dr. Kamal Kant Kohli
Published On 2021-02-12 14:07 GMT | Update On 2021-02-13 10:02 GMT
- Data from a single small RCT suggest that low-dose corticosteroids may be effective for ocular MG and may avoid side effects associated with high-dose corticosteroids.
- AChR-Ab+ patients with ocular MG who do not respond adequately to acetylcholinesterases and who either prefer not to take IS therapy or have contraindications to or are refractory to IS agents may be offered thymectomy.
- Rituximab should be considered as an early therapeutic option in patients with MuSK-Ab+ MG who have an unsatisfactory response to initial immunotherapy.
- The efficacy of RTX in refractory AChR-Ab+ MG is uncertain. It is an option if patients fail or do not tolerate other IS agents.
- Although evidence from RCTs is lacking, oral MTX may be considered as a steroid-sparing agent in patients with generalized MG who have not tolerated or responded to steroid-sparing agents that are better supported by RCT data.
- Eculizumab should be considered in the treatment of severe, refractory, AChR-Ab+ generalized MG.
- The role of eculizumab in the treatment of MG is likely to evolve over time. Until further data become available to allow comparisons of cost and efficacy with other treatments, eculizumab should be considered after trials of other immunotherapies have been unsuccessful in meeting treatment goals.
- Recommendations of the Advisory Committee on Immunization Practices or other local guidelines regarding immunization against meningococcal meningitis should be followed before treatment with eculizumab.
- Future research should include assessment of the duration of eculizumab therapy necessary to achieve and maintain treatment goals, its efficacy in other MG populations (MG with thymoma and seronegative MG), and in other stages of disease (MG crises, exacerbations, and early therapy in nonrefractory AChR-Ab+ MG).
- The risk of MG and other immune-mediated neurologic illnesses should be discussed with patients who are candidates for ICIs.
- At this time, there is no evidence to either support or refute the utility of AChR antibody testing in patients without MG before starting ICIs.
- MG associated with ICIs is generally severe, with a high rate of respiratory crises.
- Early aggressive treatment with high-dose steroids in combination with plasma exchange or IVIg may be required in patients who develop overt MG while on ICIs. The decision to withdraw ICIs is determined by the oncologic status.
- Preexisting MG does not constitute an absolute contraindication to the use of ICIs, at least in patients with well-controlled disease (MM status or better). However, in these patients:
- It may be prudent to avoid combined therapy (anti–CTLA-4 plus anti-PD-1/PD-L1 monoclonal antibodies), given the higher potential for severe irAEs.
- Close clinical monitoring, particularly of respiratory and bulbar function, is mandatory.
- Although the therapeutic response to ICIs seems to be less satisfactory in patients receiving immunosuppressants, MG treatment should be maintained and may even be restarted in patients whose MG is in remission before treatment with ICIs.
"This updated formal consensus guidance of international MG experts, based on new evidence, provides recommendations to clinicians caring for patients with MG worldwide," concluded the authors.
The guideline titled, "International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update," is published in the journal Neurology.
DOI: https://n.neurology.org/content/96/3/114
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