What is the treatment regimen for refractory myasthenia gravis (MG)?

Updated: Aug 27, 2018
  • Author: Abbas Jowkar, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

A treatment strategy using the following regimen for refractory MG, or MG presenting in crisis is as follows:

  • Do 5 PLEX sessions.
  • Start azathioprine (AZA)
  • IV methylprednisolone at dose of 500-1000 mg daily for 5 days may be used, followed by 0.8 mg/kg IV daily until patient is capable of taking medications by mouth. Start prednisone at 60 mg PO daily for 2 weeks, then 50 mg PO daily for 2 weeks, then 40 mg PO daily for a month. This is followed by a slow taper by 5 mg per month to reach 20 mg/day. Around this time (6 months), the imuran (AZA) reaches therapeutic efficacy ("kicks in"). From then on, taper prednsione 2.5 mg per month while monitoring for worsening of myasthenia symptoms. Goal is to achieve prednisone 5–7.5 mg PO daily or 10 mg every other day (no significant side effects if continued on this dose). 

Although the steroids may initially worsen the patient’s myasthenia symptoms, it is not an issue as the patient is in the ICU setting and intubated. It is hypothesized that corticosteroids have a mild neuromuscular blocking properties that precede their immunomodulatory beneficial effects.


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