Maintenance plasma exchange treatment for muscle specific kinase antibody positive myasthenia gravis patients
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Published on: May 18, 2017
Chisa Yamada1, James W Teener2, Robertson D Davenport1
1Division of Transfusion Medicine, Department of Pathology, University of Michigan, Ann Arbor, Michigan.
Background:
Anti-muscle specific kinase antibody positive myasthenia gravis (MuSK MG) is often characterized by a relatively severe and progressive course, refractoriness to standard myasthenia gravis (MG) medications, and an increased risk of myasthenic crisis. We report here successful management of three MuSK MG patients using maintenance therapeutic plasma exchange (TPE) treatment for up to 4.5 years.
Materials:
The study was a 5-year retrospective review of all MG patients treated with TPE between 2008 and 2013 at University of Michigan. Inclusion criteria of MuSK MG were positive for anti-MuSK antibodies and a diagnosis of MuSK MG by staff neurologists. Patient data included age, gender, diagnostic testing results, medications, and the dates and response to TPE treatments.
Results:
A total of 153 MG patients underwent at least one course of TPE between 2008 and 2013. A total of 12 patients (7.8%) were positive for anti-MuSK antibodies. Patients were predominantly female (83.3%) and a median age of onset was 46-years old. Three MuSK MG patients were successfully managed with maintenance TPE.
Conclusion:
Maintenance TPE may be an effective option for MuSK MG patients. The key of successful maintenance treatment at our institution has been to tailor the TPE frequency for each individual, and to modify the treatment interval in conjunction with medical management.
Insights
Maintenance therapeutic plasma exchange (TPE) offers a successful treatment option for anti-muscle specific kinase antibody positive myasthenia gravis (MuSK MG) patients, managing severe symptoms effectively.
Area of Science:
- Neurology
- Immunology
Background:
- Anti-muscle specific kinase antibody positive myasthenia gravis (MuSK MG) presents severe, progressive symptoms and resistance to standard treatments.
- MuSK MG patients face a higher risk of myasthenic crisis.
Observation:
- A retrospective review of 153 myasthenia gravis (MG) patients treated with therapeutic plasma exchange (TPE) between 2008 and 2013 was conducted.
- Twelve patients (7.8%) were diagnosed with MuSK MG, predominantly female with a median age of onset of 46.
Findings:
- Three MuSK MG patients achieved successful management through maintenance TPE.
- This treatment was sustained for up to 4.5 years in these patients.
Implications:
- Maintenance TPE is a viable and effective therapeutic strategy for managing MuSK MG.
- Individualized TPE frequency and integration with medical management are crucial for successful long-term treatment of MuSK MG.
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