Use and monitoring of low dose rituximab in myasthenia gravis

Blum, Stefan; Gillis, David; Brown, Helen; Boyle, Richard; Henderson, Robert D.; Heyworth-Smith, David; Hogan, Patrick G.; Kubler, Paul; Lander, C. M.; Limberg, Nicole; Pillans, Peter I.; Prain, Kerri; Staples, Christopher; Walsh, Michael D.; McCombe, Pamela A.; Wong, Richard

doi:10.1136/jnnp.2010.220475

Cited by 95 publications

(80 citation statements)

References 19 publications

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“…Response rates between 78% and 100% for clinical improvement have been reported over time frames typically .6 months, often with repeated cycles of treatment; in addition, concomitant reductions in corticosteroid or immunosuppressive doses were reported. [15][16][17] Similar response rates have been seen in non-treatment-refractory MG. Complication rates varied between studies, with the most common adverse events directly related to infusions (flushing, pruritus, chills, or rigors).…”

Section: Musk Phenotypesupporting

confidence: 59%

Myasthenia gravis

Statland

Ciafaloni

2013

Neur Clin Pract

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SummaryMyasthenia gravis (MG) is the most common autoimmune disease affecting neuromuscular junction transmission. MG is characterized by muscle weakness that worsens with activity and fluctuates over the course of the day. Involvement of respiratory musculature can lead to life-threatening crisis requiring intensive care unit care. Antibody testing is positive in most patients with MG. Treatment of MG includes short-term symptomatic treatment, chronic immunosuppression, surgical intervention, and immunomodulatory therapies for severe disease or crisis. We review advances in 5 areas relevant to diagnosis and management of MG: the role of IV immunoglobulin vs plasmapharesis in myasthenic crisis and severe disease; the clinical characterization of patients with antibodies to muscle-specific tyrosine kinase receptors; old and new investigational treatments; management of MG in pregnancy; and new confirmatory diagnostic tests. M yasthenia gravis (MG) is the most commonly encountered autoimmune disease of the neuromuscular junction with an estimated worldwide prevalence between 15 and 179 per million people. MG causes fluctuating weakness that worsens with activity and as the day progresses, and ocular weakness, causing ptosis and diplopia.1 In 15% of patients, life-threatening respiratory weakness can occur, called myasthenic crisis. Ocular symptoms are the most common presenting symptoms, with around two-thirds of patients progressing to generalized disease, usually within the first 2 years. The diagnosis is based on clinical history and neurologic examination and confirmed by electrodiagnostic testing and the presence of serum autoantibodies directed at proteins in the neuromuscular junction. The vast majority of patients with generalized MG (;85%) and pure ocular MG (;50%) will have antibodies to the skeletal muscle nicotinic acetylcholine receptor (AChR). An additional 8%-10% of patients with generalized disease have antibodies to muscle-specific tyrosine kinase receptor (MuSK), an enzyme involved in acetylcholine receptor clustering in the synaptic cleft.

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Section: Musk Phenotypesupporting

confidence: 59%

Myasthenia gravis

Statland

Ciafaloni

2013

Neur Clin Pract

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“…In support of this notion, there are a few reports documenting reduced rituximab dosage in myasthenia gravis and autoimmune cytopenias. 8,9 A study comparing 100-and 1,000-mg rituximab treatment suggests earlier repopulation of B cells (99 vs 184 days) and relapse in 2 patients with NMO. 4 However, these 2 patients were considered rituximab "nonresponders," which may not be associated with 100-mg rituximab treatment.…”

Section: Kinetics Of the B-cell Population In Patientsmentioning

confidence: 99%

Responsiveness to reduced dosage of rituximab in Chinese patients with neuromyelitis optica

Yang

et al. 2013

Neurology

113

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Responsiveness to reduced dosage of rituximab in Chinese patients with neuromyelitis optica ABSTRACT Objective: To determine the effect of a lower dose of rituximab in depleting B lymphocytes, maintaining low B-cell counts, and relapse in patients with neuromyelitis optica (NMO) and NMO spectrum disorders. Methods:We treated 5 Chinese patients with deteriorating NMO and NMO spectrum disorders with a 100-mg IV infusion of rituximab once a week for 3 consecutive weeks, followed by additional infusion of the same dosage depending on circulating B-cell repopulation.Results: This reduced dosage of rituximab was sufficient to deplete B cells and maintain low B-cell counts. None of the treated patients experienced relapse, and all patients exhibited stabilized or improved neurologic function during the 1-year follow-up period. MRI revealed the absence of new lesions, no enhancement in spinal cord and brain, a significant shrinkage of spinal cord segments, and a reduction/disappearance of previous brain lesions. Conclusion:

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“…Rituximab, an anti-CD20 biologic, has gained support in managing MuSK MG, as its application affects sustained clinical improvement along with a marked decline in MuSK autoantibody titer in the majority of patients (5,6). In comparison with AChR MG, fewer relapses have been reported to date in MuSK MG patients who achieved stable clinical remission after rituximab (7)(8)(9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%