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How would you treat a patient with necrotizing myopathy with positive anti-HMHCoR Ab unresponsive to standard therapies?

How would you treat a patient with necrotizing myopathy with positive anti-HMHCoR Ab (and low TIF1 gamma) that failed prednisone + Cellcept, IVIG + Cellcept, Rituximab alone, methotrexate (20mg) + IVIG (2g every 4 weeks)?
1 Answer
Mednet MemberInvited Expert
Neurology · University of Minnesota
Answered on

There are several questions raised by this scenario. I have personally never seen a patient with true HMGCR-antibody + necrotizing autoimmune myopathy fail a combination of 3 immunotherapies (high dose prednisone, IVIG, and either Rituximab or oral immunosuppression like Cellcept/azathioprine, etc), with one exception-see point #4. Therefore, I would take the following approach:

  1. Is the phenotype consistent with the diagnosis?
  2. What technique was used to determine the presence of HMGCR antibody? T...

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