Myasthenia gravis has a large inventory of medications, and these rheumatism drugs have their magical powers!

*It is only for medical professionals to read and refer to such a complete summary.
Still not writing it down? Myasthenia gravis (MG) is an autoimmune disease with acquired neuromuscular junction transmission disorder mediated by autoantibodies
.

At present, domestic drug therapy mainly uses cholinesterase inhibitors, glucocorticoids and other immunosuppressive agents
.

According to the mechanism of drug treatment, it can be divided into symptomatic treatment drugs and counter-cause treatment drugs
.

Correctly mastering the treatment points of various drugs is the key to achieving the desired effect of treatment
.

1 Cholinesterase inhibitor treatment-the most commonly used symptomatic treatment is brompistigmine, which is the first-line drug for the treatment of all types of MG, which can relieve and improve the clinical symptoms of most MG patients
.

As the first choice for the initial treatment of MG, it can be combined with hormones and other non-hormonal immunosuppression depending on the condition
.

Usage: The first dose of bropistigmine for general adults is 60mg (children use it according to the specific age), 3 to 4 times per day, and the maximum dose throughout the day does not exceed 480mg
.

According to the sensitivity of MG patients to this drug, adjust the dose and gradually reduce or stop the drug when the treatment goal is reached
.

It is also safe and effective to use this medicine during pregnancy
.

2 Immunosuppressive therapy-causal therapy includes glucocorticoids and other immunosuppressive agents, such as azathioprine (AZA), tacrolimus (FK-506), mycophenolate mofetil (MMF), cyclosporine , Methotrexate and cyclophosphamide
.

1.
Glucocorticoids are still the first-line drugs for the treatment of MG, which can significantly improve the symptoms of 70% to 80% of patients
.

Mainly oral prednisone acetate and methylprednisolone
.

Prednisone acetate is taken according to the weight of 0.
5～1.
0mg/(kg·d) in the morning, and the maximum dose does not exceed 100mg/d (the glucocorticoid dose conversion relationship is: 5mg prednisone acetate = 4mg methylprednisolone), generally 2 It takes effect within a week, and the effect is most significant in 6 to 8 weeks
.

75% mild to moderate MG has a good response to 200 mg prednisone, starting with 20 mg, increasing by 10 mg every 5 to 7 days to reach the target dose
.

After reaching the treatment goal, keep it for 6 to 8 weeks and then gradually reduce the dose, reduce 5-10 mg every 2 to 4 weeks, and reduce 5 mg every 4 to 8 weeks after reaching 20 mg.
If appropriate, take the lowest effective dose orally every other day.
If the dose is reduced too quickly, it can cause illness.
Love relapsed
.

2.
The combined use of AZAAZA and glucocorticoids can help reduce the amount of hormones and prevent the recurrence of the disease
.

AZA has a slower onset of effect, more than 3 to 6 months after taking the drug, and full effect can be achieved after 1 to 2 years, which can significantly improve the symptoms of 70% to 90% of MG patients
.

Usage: Start with a small dose, 50mg/d, increase by 50mg every 2 to 4 weeks, until the effective therapeutic dose is [Children 1~2mg/(kg·d) according to body weight, adults 2~3mg/(Kg·d), Oral 2 to 3 times]
.

The main side effects include bone marrow suppression (leucopenia, anemia and thrombocytopenia), liver damage and the like
.

If the white blood cell count is lower than 4.
0×/109L, AZA should be reduced; if the white blood cell count is lower than 3.
0×109 or the liver function test index is 3 times the upper limit of normal, the drug should be stopped immediately
.

3.
Tacrolimus The mechanism of tacrolimus is similar to that of cyclosporine.
It exerts an immunomodulatory effect by inhibiting calcineurin, with good tolerance and low nephrotoxicity
.

Tacrolimus is suitable for MG patients who cannot tolerate the side effects of hormones and other immunosuppressive agents or whose efficacy is poor, especially those with positive RyR antibodies
.

Tacrolimus has a fast onset of action, usually in about 2 weeks, and the effect is dose-dependent
.

Usage: 3.
0mg/d, orally on an empty stomach twice, or 0.
05～0.
1mg/(Kg·d) according to body weight
.

After taking the medicine or adjusting the dosage of the drug for 3 to 4 days, the blood drug concentration is screened, and the ideal trough concentration is 2-9ng/ml
.

With trough concentration ≥ 4.
8 ng/ml, 92% of patients can achieve MMS (there is no functional limitation caused by muscle weakness, some muscle weakness can be found by a professional neuromyologist) or better
.

4.
MMFMMF has the same mechanism of action as AZA, but it is safer and well tolerated.
Long-term use can make most patients reach MMS or better
.

How to use: The initial dose is 0.
5～1.
0g/d, taken orally in 2 times; the maintenance dose is 1.
0～1.
5g/d, and the reduction does not exceed 500mg/d after the symptoms are stable.
Sudden withdrawal or rapid reduction can lead to relapse and relapse.
Worsened
.

MMF cannot be used simultaneously with AZA
.

For the first 6 months after the medication, blood routine and liver and kidney function were monitored every month, and blood routine and liver and kidney function were monitored every 3 months thereafter
.

MMF is teratogenic, and it is forbidden for pregnant women or pregnant women
.

5.
Cyclosporine Cyclosporine inhibits the secretion of pro-inflammatory cytokines including interleukin 2 (IL-2) and gamma interferon by interfering with calcineurin signal, thereby exerting immunosuppressive effects.

.

Effective in 3 to 6 months, it is used for patients who have poor curative effect on hormones and AZA or cannot tolerate their side effects
.

According to body weight 2～4mg/(kg·d) orally, the plasma concentration should be monitored during use.
The recommended blood concentration is 100～150ng/ml
.

During the medication, monitor blood routine, liver and kidney function at least once a month, and closely monitor blood pressure
.

Due to the high nephrotoxicity of cyclosporine and the interaction with other drugs, it is not recommended as the first choice
.

6.
Cyclophosphamide Cyclophosphamide is used for refractory and MG with thymoma that are ineffective in the treatment of other immunosuppressive agents
.

Combined use with hormones can significantly improve the symptoms of muscle weakness and reduce the amount of hormones at 6 to 12 months
.

Usage: Adults receive 400-800mg/week by intravenous drip, or take twice orally, 100mg/d, until the total amount is 10-20g.
Individual patients need to take up to 30g
.

Children should be used with caution
.

7.
Methotrexate Methotrexate is used as a third-line drug for refractory or MG with thymoma that is ineffective in other immunosuppressive treatments
.

Oral, starting at 10mg per week, gradually increasing to 20mg/week.
If you cannot tolerate the adverse gastrointestinal reactions caused by oral preparations, intramuscular injections can also be selected.
In general, intramuscular injections can make patients tolerate higher doses
.

Folic acid 1mg/d should be added during treatment to prevent stomatitis, and side effects such as bone marrow suppression and liver damage should be paid close attention to
.

Methotrexate has reproductive teratogenicity and is contraindicated by pregnant or pregnant women
.

The drugs used with caution for 3MG patients include: anti-infective drugs, such as aminoglycoside antibiotics and antifungal drugs such as amphotericin; cardiovascular drugs such as lidocaine, quinidine, β-blockers, vera Pamir, etc.
; anti-epileptic drugs such as phenytoin, ethosuximide, etc.
; antipsychotic drugs such as chlorpromazine, lithium carbonate, diazepam, clonazepam, etc.
; narcotic drugs such as morphine, pethidine, etc.
; anti-rheumatic drugs Such as penicillamine, chloroquine and so on
.

Because these drugs may make the symptoms of MG patients worse
.

4 The latest recommendations of the American Myasthenia Gravis Foundation (MGFA) 1.
Regarding the drug treatment of ocular muscle myasthenia gravis, cholinesterase inhibitors are the first choice for symptomatic treatment of ocular muscle myasthenia gravis.
If muscle palsy causes functional limitation or annoys the patient, immunosuppressive (IS) therapy should be initiated; the initial IS treatment of ocular muscle myasthenia gravis should be treated with corticosteroids.
If a single hormone is ineffective or there is a counter-indication Or when hormones cannot be tolerated, other non-hormonal IS drugs can be added as hormone reducing agents
.

2.
The application of rituximab is not sure about the curative effect of refractory anti-muscle-specific tyrosine kinase antibody positive (AChR-Ab+) myasthenia gravis.
It can be used as the failure of other IS treatments or the inability to tolerate other IS treatments.
A treatment option at the time
.

3.
The application of Iculizumab is recommended for the treatment of severe refractory AChR-Ab + systemic myasthenia gravis
.

However, due to the lack of comparative data on the treatment cost and efficacy of iculizumab and other therapeutic drugs, the use of iculizumab should be considered when the treatment goals cannot be achieved after trying other immunotherapeutic drugs
.

4.
Application of methotrexate Due to the lack of evidence from randomized controlled trials, it is recommended that patients with systemic myasthenia gravis cannot tolerate or do not respond to steroid-reducing agents supported by the data of randomized controlled trials, consider giving it Oral methotrexate
.

References: [1] Neuroimmune Branch of the Chinese Society of Immunology.
Guidelines for the diagnosis and treatment of myasthenia gravis in China (2020 edition) [J].
Chinese Journal of Neuroimmunology and Neurology, 2021, 28 (1): 1-12.
[ 2] Neuroimmunology Group of Neurology Branch of Chinese Medical Association, Neuroimmunology Branch of Chinese Immunology Society.
Guidelines for the diagnosis and treatment of myasthenia gravis in China 2015[J].
Chinese Journal of Neurology, 2015, 48 (11): 934-940.
[3] Li Jianping.
Interpretation of "International Consensus Guidelines for the Management of Myasthenia Gravis: 2020 Update" [J].
Journal of Neurology and Neurorehabilitation, 2021, 32-38.