From the FDA Drug Label
1.3 Generalized Myasthenia Gravis (gMG) SOLIRIS is indicated for the treatment of generalized myasthenia gravis (gMG) in adult patients who are anti-acetylcholine receptor (AchR) antibody positive. The best immunomodulatory treatment for refractory Myasthenia Gravis (MG) with positive anti-AChR and striational antibodies is Eculizumab (SOLIRIS), as it is indicated for the treatment of generalized myasthenia gravis (gMG) in adult patients who are anti-acetylcholine receptor (AchR) antibody positive 1 1.
- Key points:
- Eculizumab is a complement inhibitor that can help reduce the symptoms of gMG.
- It is essential to note that patients receiving SOLIRIS are at increased risk for invasive disease caused by Neisseria meningitidis, and vaccination against meningococcal infection is recommended at least 2 weeks prior to initiation of SOLIRIS 1.
- The recommended dosage regimen for gMG is 900 mg weekly for the first 4 weeks, followed by 1200 mg for the fifth dose 1 week later, then 1200 mg every 2 weeks thereafter 1.
From the Research
For refractory Myasthenia Gravis (MG) with positive anti-AChR and striational antibodies, eculizumab is currently considered the best immunomodulatory treatment option, as it has shown superior efficacy compared to rituximab in recent studies. The recommended regimen for eculizumab is typically 900 mg weekly for 4 weeks, then 1200 mg every 2 weeks 2. Patients usually begin showing improvement within 1-3 months, with maximal benefits often seen by 6 months. For maintenance, treatment can be continued as needed to control symptoms. In addition to eculizumab, other effective options include rituximab (375 mg/m² weekly for 4 weeks, or alternatively, 1000 mg given twice, two weeks apart), mycophenolate mofetil (1000-1500 mg twice daily), or intravenous immunoglobulin (IVIG) as maintenance therapy (1-2 g/kg every 4-6 weeks) 3, 4, 5. These treatments work by targeting different aspects of the autoimmune response - eculizumab inhibits complement activation, rituximab depletes B cells that produce pathogenic antibodies, and mycophenolate suppresses lymphocyte proliferation. Before initiating these therapies, patients should undergo screening for infections like hepatitis B and tuberculosis, and vaccination status should be updated. Regular monitoring of complete blood counts and immunoglobulin levels is essential during treatment to detect potential adverse effects early. A recent real-world study has also confirmed the safety and effectiveness of eculizumab in refractory MG patients, with significant reductions in myasthenic exacerbations and crisis, and immunosuppressant-sparing benefits 6.
Some key points to consider when using eculizumab for refractory MG include:
- Close monitoring of patients for signs of infection and other adverse effects
- Regular assessment of clinical response and adjustment of treatment as needed
- Consideration of the potential for reduced immunosuppressant use and its implications for patient management
- The importance of updating vaccination status and screening for infections before initiating treatment
Overall, the choice of immunomodulatory treatment for refractory MG should be individualized based on patient-specific factors, including disease severity, medical history, and response to previous treatments. However, based on the current evidence, eculizumab is a promising option for patients with refractory MG and positive anti-AChR and striational antibodies.