A MODERN VIEW OF THE ETHIOLOGY, PATHOGENESIS, PATOMORPHOLOGY OF MYASTENIA: LITERATURE REVIEW AND CASE REPORT

Filenko B. M.

LITERATURE REVIEWS

Scentific article

Myasthenia gravis is a rare disease, but it is the most common neuromuscular disorder that has a high rate of disability and mortality. Therefore, myasthenia remains an important medical and social problem that needs to be thoroughly studied. Hereditary factors associated with the human leukocyte antigen gene play a role in the pathogenesis of myasthenia gravis. Miasthenia is a classic example of a type II hypersensitivity reaction. However, its pathogenesis depends on the form of the disease, characterized by the formation of antibodies of different subclasses of IgG for which the targets are the components of the receptor apparatus of the synapse. Identification of the source of autoantibodies is important, as antibody-producing cells are the main therapeutic targets. Antibodies are produced by antigen-specific subgroups of B-cells, that are short-and long-lived plasma cells. T-cell assistance is required for B-lymphocyte activation, so myasthenia is classified as B-cell-mediated T-cell-dependent autoimmune disease. Acetylcholine-myasthenia patients often have thymic pathology, which is confirmed by clinical improvement in acetylcholine-myasthenia patients after thymectomy. Thymus abnormalities are not detected in patients with muscle-specific kinase-myasthenia and no clinical benefit of thymectomy is observed. The role of the thymus in lipoprotein-specific protein 4-myasthenia is not established, only in the presence of additional antibodies to musclespecific kinase or lipoprotein-specific protein 4 can be observed thymus hyperplasia. This form of the disease is predominantly found in women, characterized by general muscular weakness. There are not enough morphological criteria for the diagnosis of myasthenia gravis. Therefore, the diagnosis of this disease must take into account the etiological, clinical and genetic factors. For complete morphological diagnostics it is necessary to use light-optical, histochemical, enzymohistochemical, immunomorphological and electron-microscopic methods of investigation. Here is an example of a lethal case of generalized myasthenia gravis with early onset, secondary muscle weakness in the upper and lower extremities with concomitant glomerulonephritis, discussing the pathogenetic features of these diseases. Myasthenia is rarely associated with nephropathy. The mechanism of nephrotic syndrome development is an imbalance between T-helper classes 1 and 2. In myasthenia, T-cell dysfunction leads to the production of lymphokines, which increase the permeability of the glomerular basement membrane. Doctors should look for a potential cause, such as myasthenia gravis and other thymus-related diseases, diagnosing glomerulonephritis with minimal changes in the adult.

myasthenia gravis, glomerulonephritis, pathogenesis, thymoma, thymic hyperplasia.

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«Bulletin of problems biology and medicine» Issue 4 Part 2 (154), 2019 year, 47-52 pages, index UDK [616.74+616.8]-009-091

10.29254/2077-4214-2019-4-2-154-47-52