Mūsdienīgas autoimūnu encefalītu ārstēšanas iespējas
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Date
2021
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Rīgas Stradiņa universitāte
Rīga Stradiņš University
Rīga Stradiņš University
Abstract
Autoimūna encefalīta (AE) diagnoze mūsdienās ir ļoti aktuāla un kļūst arvien biežāk sastopama ārsta neirologa ikdienā. Slimības prevalence populācijā pēdējās desmitgades laikā pakāpeniski pieaug.
Pētnieciskā darba mērķis ir apkopot un sistematizēt literatūrā pieejamo informāciju un pasaules pētījumu datus par AE medikamentozas terapijas iespējām un tās efektivitāti. Darbā papildus veikta arī viena klīniskā gadījuma aprakstoša un padziļināta analīze, izmantojot Paula Stradiņa klīniskās universitātes slimnīcas arhīvā pieejamo pacienta slimības vēsturi, kā arī iegūto rezultātu salīdzināšana ar literatūrā pieejamajiem pētījumu rezultātiem.
Nozīmīga ir ne tikai slimības savlaicīga diagnostika, bet arī agrīni uzsākta imūnterapija, jo tai pastāv saistība ar labāku slimības iznākumu kā arī neiroloģiskā stāvokļa uzlabošanos. Lai gan pastāv plašas medikamentozas ārstēšanas iespējas, pielietojot dažādu imūnterapiju, precīzas terapijas vadlīnijas slimības ārstēšanai nav izstrādātas un tā tiek individuāli piemērota katram pacientam.
Medikamentozajā ārstēšanā plaši tiek pielietoti gan imūnsistēmu supresējoši medikamenti, gan arī tādi, kuri mērķēti tiek vērsti uz slimības patoģenēzes procesā iesaistītajām antivielām. Pirmās izvēles ārstēšanas shēmās galvenokārt tiek iekļauti glikokortikosteroīdi, intrevenozs imūnglobulīns un plazmaferēze. Otrās rindas medikamentoza terapija (Rituximab, Cyclophosphamide) tiek pielietota gadījumos, kad izmantojot pirmās izvēles terapiju, nav vērojama klīniska uzlabošanās, kā arī ir novērojama smaga slimības gaita un recidīvi. Rituximab ir monoklonāla antiviela, kura novērš plazmocītu un B limfocītu mediēto imunoloģisko procesu, savukārt Cyclophosphamide samazina limfocītu proliferāciju. Specifisku autoimūnu un iekaisuma procesa citokīnu produkcijas nomākšanai, terapijā pielieto arī Bortezomib, zemas devas interleikīnu 2 (IL-2) un monoklonālo antivielu Tocilizumab.
Pētnieciskajā darbā aprakstītajā klīniskajā gadījumā tiek novērota strauja AE progresija. Neskatoties uz to, ka tikusi pielietota multimodāla terapija un izmantoti gan pirmās izvēles medikamenti Methylprednisolone un intervenozais imūnglobulīns, gan arī otrās izvēles medikaments Cyclophosphamide, iestājas pacienta exitus letalis.
The diagnosis of autoimmune encephalitis (AE) is very relevant today and is becoming more common in the daily life of a neurologist. The prevalence of the disease across the population has been gradually increasing over the last decade. The aim of this research is to compile and systematize the information available in the literature and world research data on the possibilities and effectiveness of AE drug therapy. In addition, a descriptive and in-depth analysis of one clinical case was performed during this research using a patient's medical history available in the archives of Pauls Stradins Clinical University Hospital, and a comparison was made with the obtained results within the research and results available in the literature. Not only an early diagnosis of the disease is important, but also an early initiation of the immunotherapy, which is associated with a better outcome of the disease as well as an overall improvement in the neurological condition. Although there is a wide range of drug treatments available within a variety of immunotherapies, precise treatment guidelines for the disease have not been developed and are tailored to each patient. Both immunosuppressive drugs and those that specifically target antibodies involved in the pathogenesis of the disease are widely used in drug therapy. Preferred treatment regimens mainly include glucocorticosteroids, intravenous immunoglobulin and plasmapheresis. Second-line drug therapy (Rituximab, Cyclophosphamide) is used in cases where there is no clinical improvement with first-line therapy, as well as severe disease progression and relapse is observed. Rituximab is a monoclonal antibody that suppresses the immunological process mediated by plasma cells and B lymphocytes, while Cyclophosphamide reduces lymphocyte proliferation. Bortezomib, low-dose interleukin 2 (IL-2) and the monoclonal antibody Tocilizumab are also used to suppress the production of specific autoimmune and inflammatory cytokines. In the clinical case described in this research, rapid progression of AE is observed. Despite the use of multimodal therapy and the use of both the first-line drug Methylprednisolone and the interventional immunoglobulin and the second-line drug Cyclophosphamide, exitus letalis occurs in the respective patient.
The diagnosis of autoimmune encephalitis (AE) is very relevant today and is becoming more common in the daily life of a neurologist. The prevalence of the disease across the population has been gradually increasing over the last decade. The aim of this research is to compile and systematize the information available in the literature and world research data on the possibilities and effectiveness of AE drug therapy. In addition, a descriptive and in-depth analysis of one clinical case was performed during this research using a patient's medical history available in the archives of Pauls Stradins Clinical University Hospital, and a comparison was made with the obtained results within the research and results available in the literature. Not only an early diagnosis of the disease is important, but also an early initiation of the immunotherapy, which is associated with a better outcome of the disease as well as an overall improvement in the neurological condition. Although there is a wide range of drug treatments available within a variety of immunotherapies, precise treatment guidelines for the disease have not been developed and are tailored to each patient. Both immunosuppressive drugs and those that specifically target antibodies involved in the pathogenesis of the disease are widely used in drug therapy. Preferred treatment regimens mainly include glucocorticosteroids, intravenous immunoglobulin and plasmapheresis. Second-line drug therapy (Rituximab, Cyclophosphamide) is used in cases where there is no clinical improvement with first-line therapy, as well as severe disease progression and relapse is observed. Rituximab is a monoclonal antibody that suppresses the immunological process mediated by plasma cells and B lymphocytes, while Cyclophosphamide reduces lymphocyte proliferation. Bortezomib, low-dose interleukin 2 (IL-2) and the monoclonal antibody Tocilizumab are also used to suppress the production of specific autoimmune and inflammatory cytokines. In the clinical case described in this research, rapid progression of AE is observed. Despite the use of multimodal therapy and the use of both the first-line drug Methylprednisolone and the interventional immunoglobulin and the second-line drug Cyclophosphamide, exitus letalis occurs in the respective patient.
Description
Medicīna
Medicine
Veselības aprūpe
Health Care
Medicine
Veselības aprūpe
Health Care
Keywords
Neiroloģija, autoimūns encefalīts, imūnterapija, Neurology, autoimmune encephalitis, immunotherapy