Myasthenia Gravis and Related Disorders by Henry J. Kaminski & Linda L. Kusner

Author:Henry J. Kaminski & Linda L. Kusner
Language: eng
Format: epub
Publisher: Springer International Publishing, Cham

Rituximab

Rituximab is a monoclonal antibody directed against CD20 cells. Retrospective evaluations have led to great enthusiasm for application of the agent for treatment-resistant MG [121–124]. A blinded, prospective review for MuSK MG supports rituximab improves disease severity and limits use of other MG treatments [125]. A phase II trial for ACHR and MuSK-positive MG is ongoing at the time of this writing. Dosing of rituxan is typically a once a week regimen for 4 weeks at a dose of 375 mg/m2 of body surface. Pretreatment with acetaminophen, diphenhydramine, corticosteroid, or a combination is given by some investigators. Repeat dosing may be given in 6 months as is being performed in the phase II trial, or patients have been monitored for recurrent weakness and then repeat treatment given. Infusion reactions of varying severity consisting of fever, nausea, headache, pruritus, headache, and angioedema occur during infusion and may improve with pretreatment and slowing the infusion rate. Severe infections, including progressive multifocal leukoencephalopathy, may occur. Activation of latent hepatitis B infection may occur.

Mechanism of Action. Rituxan removes CD20-expressing cells, which include activated B cells and pre-B cells. These cells serve to produce pro-inflammatory cytokines, present antigen, and provide T cell support. The reduction of CD20 cells impacts these properties, and the reduction of antibody-producing cells is likely a mechanism that reduces MG severity [126, 127].

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