Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity

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  • Authors: Nasonov E.L.1,2, Reshetnyak T.M.1,3, Alekberova Z.S.1
  • Affiliations:
    1. ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»
    2. ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)
    3. ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава России
  • Issue: Vol 92, No 5 (2020)
  • Pages: 4-14
  • Section: Editorial
  • URL: https://ter-arkhiv.ru/0040-3660/article/view/34518
  • DOI: https://doi.org/10.26442/00403660.2020.05.000697
  • Cite item

Abstract


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.


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About the authors

Evgeny L. Nasonov

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»; ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)

Author for correspondence.
Email: nasonov@irramn.ru
ORCID iD: 0000-0002-1598-8360

Russian Federation, Москва

акад. РАН, д.м.н., проф., науч. рук., проф.

Tatyana M. Reshetnyak

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»; ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава России

Email: nasonov@irramn.ru
ORCID iD: 0000-0003-3552-2522

Russian Federation, Москва

д.м.н., рук. лаб. сосудистой ревматологии, проф. каф. ревматологии

Zemfira S. Alekberova

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»

Email: nasonov@irramn.ru
ORCID iD: 0000-0002-6619-718X

Russian Federation, Москва

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»

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