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June is the Myasthenia Gravis (MG) Awareness Month. The Myasthenia Gravis Foundation (MGFA) sponsored an MG Awareness Month to help people understand the challenges and opportunities faced by people diagnosed with MG and their caregivers. Various actions are being taken to raise awareness.

MG is a rare autoimmune and neuromuscular disease that causes patients to experience extreme fatigue and severe muscle weakness. In Japan, clinical practice guidelines were revised in 2022, including new classifications of MG, treatment algorithms for each classification, and descriptions of complement (C5) inhibitors. Several new therapeutic drugs have been released since the guidelines were revised, and future changes in MG treatment are expected.

CareNet and Macromil CareNet conducted a survey in conjunction with MG Awareness Month among 49 CareNet neurologist members who are involved in the treatment of generalized myasthenia gravis (gMG) of moderate or high severity. Consequently, the following points were clarified.

<Survey period> 2024/3/8

  • The average number of patients with gMG of moderate or high severity that have been treated by a primary care physician in the past year was 5.9, with moderate severity accounting for 52% of the total cases.
  • Among patients with moderate to severe disease, the percentage of EFT/FT and biological agents used was the highest for “immunoglobulin therapy” and “steroid pulse therapy” at an average of 35%, followed by “biological agents” at 24%. biological agents were used in approximately 20% of patients, regardless of whether they were seriously ill. *Early fast-acting treatment (EFT)
  • Satisfaction rate (Top 2 box) for treatment of moderate or severe gMG remained at 16%. The reason for the low satisfaction level is thought to be that, although biological drugs have been added to the options, there are still patients who do not experience the effects.
  • 65% of physicians answered that the timing of using biological drugs was “after performing EFT/FT once or twice,” while 10% of physicians answered “before performing EFT/FT.” Regarding future usage policy, 31% of physicians answered “use it before performing EFT/FT.”
  • It is expected that increasing the number of options for biological drugs and using them at an early stage, as well as providing treatments tailored to the severity of the disease, will lead to improvements in current dissatisfaction and treatment satisfaction.

The average number of patients with gMG of Class III or higher (moderate or high severity) that the responding physicians had treated as their attending physicians in the past year was 5.9, with 3.1 (52%) in Class III, 1.9 (33%) in Class IV, and 0.9 (15%) in Class V.

[Table 1] Number of patients with gMG in the past year

For patients with gMG of Class III or higher (moderate or high severity), the average percentage of patients receiving each treatment (EFT/FT and biological agents) was 35% for “immunoglobulin therapy” and “steroid pulse therapy.” The most common answer was “biological products,” followed by “biological products” at 24%.

*Early fast-acting treatment (EFT)

Looking at the differences in treatment depending on the presence or absence of severe illness, physicians who treated seriously ill patients (Class IV or higher) performed “steroid pulse therapy” and “immunoglobulin therapy” on 40%. The implementation rate was higher than that of physicians who only treated patients with Class III illness. Conversely, biological agents were used in approximately 20% of patients, regardless of whether they were seriously ill.

[Table 2] Proportion of each treatment in patients with moderate or high severity gMG

When asked, “When using biological agents to treat gMG, please tell us the order of selection of drugs.” The first choice was “Wifgart,” which was the most popular choice at 43%, followed by “Soliris” (31%) and “Ultomiris” (16%). As for the second choice, “Ultomiris” was the most popular at 33%.

[Table 3] Expected selection order of biological products

When asked, “What characteristics are important when selecting a biologic agent for the treatment of gMG?” The most common answer was “strong symptom improvement effect” at 78%, followed by “Less side effects (high safety)”(51%). The response rate for “improving QOL” and “achieving MM-5 mg,” which are recommended as the basic concept for treatment in the “Myasthenia Gravis Treatment Guidelines 2022,” was 30% to 40%. First, emphasis was placed on its effectiveness in improving symptoms and safety.

*MM-5mg: Condition where the prednisolone (steroid) dose is less than 5 mg/day

[Table 4] Characteristics to consider when selecting biological products

When asked “How satisfied are you with the treatment for moderate or high severity gMG?” half of the physicians answered “satisfied” (Top 3 box). However the percentage of Top 2 boxes with high satisfaction remained at 16%.

The reason for the low satisfaction level is thought to be that, although biological drugs have been added to the options, there are still patients who do not experience the effects. However, the level of dissatisfaction decreased as the symptoms become more controllable.

[Table 5] Satisfaction with treatment of moderate or severe gMG

When asked, “When do you use biologics in the treatment of gMG?” regarding the , the most common answer was “use it after performing EFT/FT once or twice” (65%), followed by “use it before performing EFT/FT” (10%).

Regarding future usage policy, 31% of physicians answered “use it before performing EFT/FT.” It is expected that increasing the number of options for biological drugs and using them at an early stage, as well as providing treatments tailored to the severity of the disease, will lead to improvements in current dissatisfaction and treatment satisfaction.

[Table 6] When to use biologics

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