What is the principle of treating myasthenia gravis?
Let’s review why we get myasthenia gravis, because the thymus gland, as a terrorist training base, has been sending out some destructive combat units, that is, bad immune cells that are activated. These combat units will produce terrorists, i.e. auto-antibodies, and when they reach the local neuromuscular junction, they will do damage and destroy the food on our side.
In the treatment, we can understand that, firstly, the old nest can be eliminated, that is, the terrorist training base is destroyed, and the thymus gland is removed. Second, to remove and destroy its combat units, these tanks, armored vehicles, is to suppress or remove the immune cells, and third, to kill the terrorists directly, to take them all out, called the removal of autoantibodies. Fourth, as our side to increase food, extend supply lines, and relatively increase acetylcholine. So from all four aspects of myasthenia gravis can come a treatment.
At present, the main treatment methods include.
The first approach is to reduce the degradation of acetylcholine. This is actually a way to increase the grain, which is to use pyridostigmine or neostigmine to relatively increase the action of acetylcholine in the neuromuscular junction, so that the patient feels a little bit more powerful.
The second approach is to suppress the immune cells. This is the equivalent of destroying the combat unit, removing and suppressing the bad immune cells, including glucocorticoids, azathioprine, tacrolimus, cyclophosphamide, cyclosporine, enzyme phenolate, and rituximab, to name a few.
The third method is to kill the terrorist outright and get rid of it. The removal of antibodies, including human immunoglobulins, plasma replacement, immunosorbent, immunoglobulins have a structure called Fc segment, for this Fc segment can be designed some missiles, this missile we call monoclonal antibodies, missiles through the bomb-making system and Fc segment binding, as antibodies to remove the bad immunoglobulins.
The fourth method is to destroy this terrorist training base, called thymectomy. Thymoma is recommended to be removed directly, and what about patients with thymic hyperplasia, or patients with incomplete thymic degeneration? Our internists generally recommend thymectomy for patients who are positive for acetylcholine receptor antibodies, systemic, young, short in duration, and who do not respond well to cholinesterase inhibitors.
Precautions for immunotherapy
The process of immunotherapy is very long and requires rational understanding by patients, including the following aspects.
Early diagnosis, early treatment, adequate dosage and adequate course of treatment.
We often encounter patients who, after 1-2 months of standardized treatment, almost approach normal and say that I am well, so they stop all the drugs. As a result, a few weeks or months after stopping the medication, the disease is obviously recurrent, rapidly progressing, and even to the critical condition, the patient to regret it is too late. Therefore, it is very important to have adequate dosage and full course of treatment.
Also, it is important to cooperate with the doctor for regular evaluation, and if the treatment is ineffective or there are intolerable side effects, stop the corresponding therapeutic drugs as soon as possible and seek further help from the doctor.
There are individual differences in patient response to medications, and no generalizations can be made. We say that the efficacy of drugs is different for each person, so we cannot rigidly use the same type of treatment for different patients. Instead, we have to decide what the treatment plan is based on the patient’s response in the dynamic process of interaction with the patient. The space of immunotherapy depends on the fluctuation of the patient’s disease, and the more the disease fluctuates, the more effective the treatment will be. Some patients tell you that I have not changed much for 4-5 years, and that’s it. Such patients may have little benefit from treatment, and it has become irreversible damage.
In addition, treatment needs to take into account various medical and social factors.
For example, if a 90-year-old person has oculomotor myasthenia gravis, does he still need to be treated with active intervention? This is not necessarily the case, because it is necessary to take into account the other states of his body, so it is enough to be able to barely control his condition and slightly improve his symptoms. There is no need for very aggressive immunosuppressive therapy, because the risks associated with the treatment, may be greater than the benefits would be. So immunotherapy needs to have these treatment considerations.
Duration of onset of action of several treatments
Patients can become anxious during the course of their medication and repeatedly ask why they haven’t improved. When will the treatment take effect? To reduce anxiety, it is important to have a general understanding of the onset of action of some common treatments.
Pyridostigmine is fast, usually 30-45 min; plasma exchange 1 day-2 weeks; intravenous propecia 1 day-4 weeks; prednisone 2-8 weeks; azathioprine 3-18 months; cyclosporine 2-6 months; tacrolimus 1-3 months; mycophenolate 2-6 months. If we know the basic onset of action of these drugs, we will not be anxious and fearful, and will be able to cooperate with the doctor, establish a concept of living with the disease under the guidance of the doctor, fight it calmly and wait for the efficacy of the treatment.
Precautions for high-dose hormone therapy
Hormones are the most common drugs used to treat myasthenia gravis, but there are some precautions that need to be taken when using high doses.
On the one hand, hormones are by far the most effective drug, with an improvement rate of more than 90%. The initial improvement time after medication is about 2 weeks, and the average time to reach maximum improvement may take up to 6 months.
Exacerbation with high doses of hormones is relatively common, with 20-50% of people experiencing a sudden exacerbation after high doses of hormones. Some patients don’t understand why they will get worse. Probably less than 10% of people will experience this phenomenon. The exacerbation usually occurs within a week, so you can have a prediction of your situation.
Common misconceptions about the treatment of myasthenia gravis
Myasthenia gravis is a chronic disease, and many patients have some misconceptions in the treatment process. One of the most common misconceptions is that the treatment is irregular, adding or subtracting drugs as they wish. I think I am fine now, so I will not take it or take it less. Myth No. 2 is that I will be cured after having thymus surgery. Patients say that if I get the surgery done, I should be cured and I can stop taking all my medications. In fact, thymus surgery and medication for myasthenia gravis are parallel and complementary. Before preparing for thymic surgery, patients with generalized myasthenia gravis need strict medication to stabilize their disease. Long-term follow-up with an internist is also required after the surgery is completed. Myth 3: There is a “magic pill” that can be used once. I’ll take a pill that will cure me in a week. This is a misconception that patients often fall for. When you see a special treatment on the Internet, you may be fooled if you believe in it, but there is no such “magic pill” that can be used to get well. The fourth misconception is that you can stop taking medication when your symptoms improve significantly. Significant improvement only means that the disease is under control, but this disease needs long-term treatment. Is hormone treatment immediately after diagnosis? Let’s look at a patient, a male, very young, 12 years old, who has oculomotor myasthenia gravis, when he first came to the clinic, did he need to add hormone therapy? Here are four choices, you can choose, guess, as a doctor, which one should I choose? I would choose C. I would not start with hormone therapy, I would start with oral pyridostigmine and follow up with prednisone as appropriate. Because about 1/3-1/4 of adolescent patients with oculomotor type, it will spontaneously resolve, it can resolve on its own. So maybe with a little pyridostigmine, it will get better on its own later on, without adding hormones.
Treatment strategies for myasthenia gravis
We conclude with a roadmap and protocol for the treatment of myasthenia gravis. If the diagnosis of myasthenia gravis is clear, pyridostigmine can be used. If it does not work or if the disease progresses, add hormones, azathioprine along with something to counteract the side effects of hormones. If it doesn’t work again, change to other immunosuppressive drugs.
The patient needs a CT of the thymus. If there is a thymoma, thymectomy is recommended.
If there is no thymoma, but the patient is antibody positive, young and systemic, with a short course of disease, and cholinesterase inhibitors are not effective, thymectomy is still recommended.
You can make reference to such a flow chart for your own condition.
To summarize, myasthenia gravis is a rare disease and is a treatable autoimmune disease.
Diagnosis requires a comprehensive judgment by an experienced physician and cannot be based on a single laboratory test. Remember the mantra of early self-identification of the condition.
Early diagnosis and treatment is important, with adequate dosage and full course of treatment.
Build confidence and readiness to live with chronic diseases and do battle for a long time.
