Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity
Uncontrolled hypercoagulation and inflammation (thromboinflammation), which are both independent and closely related and amplifying each other pathological processes, form the basis for pathogenesis of a wide range of diseases and complications, including immuno-inflammatory (autoimmune) rheumatic d...
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| Format: | Article |
| Language: | Russian |
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"Consilium Medicum" Publishing house
2020-06-01
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| Series: | Терапевтический архив |
| Subjects: | |
| Online Access: | https://ter-arkhiv.ru/0040-3660/article/viewFile/34518/pdf |
| _version_ | 1818598009360875520 |
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| author | Evgeny L. Nasonov Tatyana M. Reshetnyak Zemfira S. Alekberova |
| author_facet | Evgeny L. Nasonov Tatyana M. Reshetnyak Zemfira S. Alekberova |
| author_sort | Evgeny L. Nasonov |
| collection | DOAJ |
| description | Uncontrolled hypercoagulation and inflammation (thromboinflammation), which are both independent and closely related and amplifying each other pathological processes, form the basis for pathogenesis of a wide range of diseases and complications, including immuno-inflammatory (autoimmune) rheumatic diseases, with the development of potentially fatal injuries of internal organs. Thrombotic microangiopathy is one of the most prominent prototypes of thromboinflammatory pathological conditions. The close link between environmental factors, hemostasis genetic defects and the complement system, inflammation and autoimmunity as pathogenetic mechanisms of microthrombosis draws particular attention to studying thrombotic microangiopathy in immuno-inflammatory rheumatic diseases, primarily systemic lupus erythematosus, antiphospholipid syndrome and scleroderma renal crisis. In future, these studies may be important for expanding the idea of the role of autoimmune mechanisms in pathogenesis of critical hemostasis disorders in human diseases, and for developing new approaches to therapy. Recently, special attention has been paid to the treatment of systemic lupus erythematosus and antiphospholipid syndrome with eculizumab, which is humanized monoclonal IgG2/4k antibody that blocks the complement component C5a and the membrane attack complex (C5b-9) formation, and which is registered for the treatment of atypical hemolytic uremic syndrome, paroxysmal nocturnal hemoglobinuria, as well as severe forms of myasthenia gravis and neuromyelitis optica. Further studies in this direction will create prerequisites for improving the prognosis not only in patients with orphan disorders, but also for widespread human diseases. |
| first_indexed | 2024-12-16T11:56:53Z |
| format | Article |
| id | doaj.art-1bd5e817c1144fe1ab4d91bbb7e0689f |
| institution | Directory Open Access Journal |
| issn | 0040-3660 2309-5342 |
| language | Russian |
| last_indexed | 2024-12-16T11:56:53Z |
| publishDate | 2020-06-01 |
| publisher | "Consilium Medicum" Publishing house |
| record_format | Article |
| series | Терапевтический архив |
| spelling | doaj.art-1bd5e817c1144fe1ab4d91bbb7e0689f2022-12-21T22:32:33Zrus"Consilium Medicum" Publishing houseТерапевтический архив0040-36602309-53422020-06-0192541410.26442/00403660.2020.05.00069731065Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunityEvgeny L. Nasonov0Tatyana M. Reshetnyak1Zemfira S. Alekberova2ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»; ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»; ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава РоссииФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»Uncontrolled hypercoagulation and inflammation (thromboinflammation), which are both independent and closely related and amplifying each other pathological processes, form the basis for pathogenesis of a wide range of diseases and complications, including immuno-inflammatory (autoimmune) rheumatic diseases, with the development of potentially fatal injuries of internal organs. Thrombotic microangiopathy is one of the most prominent prototypes of thromboinflammatory pathological conditions. The close link between environmental factors, hemostasis genetic defects and the complement system, inflammation and autoimmunity as pathogenetic mechanisms of microthrombosis draws particular attention to studying thrombotic microangiopathy in immuno-inflammatory rheumatic diseases, primarily systemic lupus erythematosus, antiphospholipid syndrome and scleroderma renal crisis. In future, these studies may be important for expanding the idea of the role of autoimmune mechanisms in pathogenesis of critical hemostasis disorders in human diseases, and for developing new approaches to therapy. Recently, special attention has been paid to the treatment of systemic lupus erythematosus and antiphospholipid syndrome with eculizumab, which is humanized monoclonal IgG2/4k antibody that blocks the complement component C5a and the membrane attack complex (C5b-9) formation, and which is registered for the treatment of atypical hemolytic uremic syndrome, paroxysmal nocturnal hemoglobinuria, as well as severe forms of myasthenia gravis and neuromyelitis optica. Further studies in this direction will create prerequisites for improving the prognosis not only in patients with orphan disorders, but also for widespread human diseases.https://ter-arkhiv.ru/0040-3660/article/viewFile/34518/pdfthrombotic microangiopathysystemic lupus erythematosusantiphospholipid syndromeeculizumab |
| spellingShingle | Evgeny L. Nasonov Tatyana M. Reshetnyak Zemfira S. Alekberova Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity Терапевтический архив thrombotic microangiopathy systemic lupus erythematosus antiphospholipid syndrome eculizumab |
| title | Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity |
| title_full | Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity |
| title_fullStr | Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity |
| title_full_unstemmed | Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity |
| title_short | Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity |
| title_sort | thrombotic microangiopathy in rheumatology a link between thrombosis and autoimmunity |
| topic | thrombotic microangiopathy systemic lupus erythematosus antiphospholipid syndrome eculizumab |
| url | https://ter-arkhiv.ru/0040-3660/article/viewFile/34518/pdf |
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