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Intravenous immunoglobulin is used in the treatment of autoimmune diseases in general, and autoimmune neuromuscular diseases in particular, including immune-mediated central and peripheral nervous system disorders. Among other treatments, due to its high anti-inflammatory properties, immunoglobulins have been used for more than three decades and are considered a well-tolerated, effective modality for autoimmune neuromuscular diseases. .
1. Overview of Autoimmune Neuromuscular Diseases and Immunoglobulins
Autoimmune neuromuscular diseases are diseases that damage nerves and muscles through the formation of autoimmune diseases. This group of diseases has a very high morbidity and disability, accounting for about half of all patients with evidence of a general autoimmune disorder.
Although each individual has different immune system abnormalities, the most common autoimmune nerve lesions are Guillain-Barre syndrome, chronic polyneuropathy, and motor neurone disease. multifocal movement and demyelinating peripheral neuropathy due to paraproteinemia.
For autoimmune neuromuscular junction diseases, those with high prevalence are myasthenia gravis, Lambert-Eaton myasthenic syndrome. For diseases that damage muscles, the most common patients are polymyositis, dermatomyositis and inclusion body myositis. In addition, certain structures of the central nervous system can also be affected by autoantibodies, causing the common disease multiple sclerosis and stiff man syndrome.
With today's medical progress in elucidating the immunogenicity of these diseases and thereby building the therapeutic foundation, immunoglobulins have been considered as a way to treat mental illnesses. A formal autoimmune neuromuscular disease, it is safe and an effective long-term therapy.
By neutralizing autoantibodies and altering inflammatory responses, immunoglobulins have somewhat limited the rate of disease progression, prevented further neuromuscular damage, and improved symptoms and prolongation of the disease. quality of life until there are truly more radical treatments.
Bệnh thần kinh cơ tự miễn gây tổn thương cơ và các dây thần kinh
2. General mechanism of immunoglobuline activities in the treatment of autoimmune neuromuscular disease
There is a great deal of evidence that immunoglobulins by different mechanisms work together to inhibit damage in the pathogenesis of autoimmune neuromuscular diseases. These mechanisms may include:
2.1. Inhibition of autoantibodies
Immunoglobuline molecules act as nonspecific antibodies, neutralizing disease-causing autoantibodies.
From there, immunoglobulins will gradually block the effect of autoantibodies on their specific organ tissues, which is the pathogenic mechanism of one of the most common autoimmune neuromuscular diseases. Guillain-Barre syndrome.
2.2. Inhibits complementary bonds and prevents the formation of membrane attack complexes
The effect of immunoglobulins on the inhibition of complement binding in the autoimmune system has been demonstrated in vitro in animal models and also in patients receiving Immunoglobuline. Immunoglobuline's mechanism of action is to inhibit the uptake of C3 and C4 into the endothelium of cells.
This is particularly significant in patients with dermatomyositis, where the pathogenesis is due to the deposition of complexes that attack the endothelial cell membrane.
2.3. Blockade of Fc receptors on macrophages
The role of macrophages is phagocytosis, i.e. cell-mediated immune activity, causing tissue damage. When immunoglobulins are infused into the systemic circulation, they immediately bind via their Fc region to the Fcγ receptors on macrophages.
As a result, the signaling system that mediates a cascade of inflammatory responses or other immune-mediated functions is suppressed.
2.4. Inhibits pathogenic cytokines and other immunoregulatory molecules
In vitro and in vivo studies have shown that immunoglobulins can help downregulate tissue expression levels or reduce circulating levels of cytokines and adhesion molecules.
From there, this effect becomes an upstream effect in the course of the immune compromise of the disease.
3. How to use immunoglobulins in the treatment of autoimmune neuromuscular diseases
Therapeutic doses of intravenous immunoglobulins have been determined empirically at 2 grams per kilogram of patient body weight.
Although the previous treatment was to divide the total dose required over five days at a daily dose of 400 mg/kg, the current preferred dosing regimen is to divide the total dose into two doses of 1 g/kg each day. times in patients who are young and have normal renal and cardiovascular function.
The basis for this change of administration is based on numerous reports that two-day infusion of immunoglobulins did not cause more adverse reactions than five-day infusion, except in patients with cardiovascular disease or high risk. Even, infusion of high doses of immunoglobulins in a short period of time results in a better response to treatment.
Loại thuốc này có tác dụng tích cực trong điều trị bệnh thần kinh cơ tự miễn
4. Things to monitor during the use of immunoglobulins
The infusion of immunoglobulins in the treatment of autoimmune neuromuscular diseases or other immunological diseases may cause undesirable effects. Therefore, during infusion, it is necessary to closely monitor the patient for the following reactions:
4.1. Changes in serum viscosity and risk of thrombosis
Suspensions of immunoglobulins used in parenteral therapy are highly viscous and may increase serum viscosity. This should be of particular concern in patients with pre-existing high serum viscosity factors, such as those with hemoglobinopathy, hypercholesterolemia, or hyperglycemia.
If serum viscosity is too high, it increases the risk of thrombotic events and can cause acute cerebral stroke, pulmonary embolism or myocardial infarction.
4.2. Migraine
In patients with a history of migraine, or migraine headache, therapy with immunoglobulins may cause an acute attack. Fortunately, however, this migraine can be prevented by giving the patient a prophylactic dose of propranolol beforehand.
In addition, the occurrence of aseptic meningitis has also been reported in patients with a history of migraine during infusion of immunoglobulins. At the same time, immunoglobulins are also being implicated in stroke in young women with a history of migraine.
4.3. Aseptic meningitis
Aseptic meningitis is considered a side effect in some patients treated with immunoglobulins. This condition is not related to Immunoglobuline preparation, rate of infusion or previous medical history.
When aseptic meningitis is encountered following Immunoglobuline infusion, symptoms will respond to strong analgesics and will subside over 24 to 48 hours. At the same time, additional diagnostic tests are rarely necessary.
4.4. Other side effects
Similar to other biological products, after infusion of Immunoglobuline, patients may experience allergic symptoms such as skin reactions or more severe anaphylactic reactions, acute kidney damage...
In summary, Immunoglobulin therapy has long been recognized as effective in autoimmune neuromuscular diseases, especially when targeted therapies are not yet available. With the above dose level and method of use, immunoglobulins play an important role in slowing the progression of the disease, limiting damage and maintaining organ system function for patients.
Autoimmune neuromuscular disease causes many serious complications, which cannot be subjective. Therefore, you should treat actively and thoroughly as soon as there are signs. To register for examination and treatment at Vinmec International General Hospital, you can contact Vinmec Health System nationwide, or register online HERE
Reference: Nhia.org; sciencedirect.com; researchgate.net; ncbi.nlm.nih.gov; impe-qn.org.vn
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