{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T00:11:19Z","timestamp":1782951079654,"version":"3.54.5"},"reference-count":88,"publisher":"Consilium Medicum","issue":"5","license":[{"start":{"date-parts":[[2020,6,5]],"date-time":"2020-06-05T00:00:00Z","timestamp":1591315200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Terapevticheskii arkhiv"],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.26442\/00403660.2020.05.000697","type":"journal-article","created":{"date-parts":[[2020,6,29]],"date-time":"2020-06-29T14:05:35Z","timestamp":1593439535000},"page":"4-14","source":"Crossref","is-referenced-by-count":19,"title":["Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity"],"prefix":"10.26442","volume":"92","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1598-8360","authenticated-orcid":false,"given":"Evgeny L.","family":"Nasonov","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3552-2522","authenticated-orcid":false,"given":"Tatyana M.","family":"Reshetnyak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6619-718X","authenticated-orcid":false,"given":"Zemfira S.","family":"Alekberova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"18344","published-online":{"date-parts":[[2020,6,5]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1159\/000322064"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-11-882993"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.3389\/fcell.2020.00073"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.11613\/BM.2012.006"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.rdc.2018.06.010"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1410490"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1016\/j.mcna.2016.09.010"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.00620117"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1093\/ckj\/sfz040"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1111\/1744-9987.12763"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(17)30062-4"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1111\/trf.14804"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.00337"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1053\/j.ackd.2019.08.012"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.00449"},{"key":"ref16","doi-asserted-by":"crossref","unstructured":"Zanatta E, Polito P, Favaro M, et al. Therapy of scleroderma renal crisis: State of the art. Autoimmun Rev. 2018;17(9):882-9. doi: 10.1016\/ j.autrev.2018.03.012","DOI":"10.1016\/j.autrev.2018.03.012"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1002\/art.40377"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1038\/nrrheum.2017.125"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1111\/imcb.12324"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1016\/j.smim.2019.101339"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019"},{"key":"ref22","doi-asserted-by":"publisher","DOI":"10.1038\/nrdp.2016.39"},{"key":"ref23","unstructured":"\u041d\u0430\u0441\u043e\u043d\u043e\u0432 \u0415.\u041b. \u0421\u0438\u0441\u0442\u0435\u043c\u043d\u0430\u044f \u043a\u0440\u0430\u0441\u043d\u0430\u044f \u0432\u043e\u043b\u0447\u0430\u043d\u043a\u0430. \u0412 \u043a\u043d.: \u0420\u043e\u0441\u0441\u0438\u0439\u0441\u043a\u0438\u0435 \u043a\u043b\u0438\u043d\u0438\u0447\u0435\u0441\u043a\u0438\u0435 \u0440\u0435\u043a\u043e\u043c\u0435\u043d\u0434\u0430\u0446\u0438\u0438. 2017. \u041c.: \u0413\u042d\u041e\u0422\u0410\u0420-\u041c\u0435\u0434\u0438\u0430, 2017; \u0441. 113-41 [Nasonov EL. Systemic lupus erythematosus. In: Russian Kliscientific recommendations. 2017. Moscow: GEOTAR-Media, 2017; p. 113-41 (In Russ.)]."},{"key":"ref24","doi-asserted-by":"crossref","unstructured":"Couture P, Hie M, Pineton de Chambrun M, et al. FRI0302 Systemic lupus erythematosus-associated thrombotic microangiopathies in 60 patients: clinical features, prognosis and treatment in a french multicentric cohort. Ann Rheum Dis. 2018;77:688-9.","DOI":"10.1136\/annrheumdis-2018-eular.6871"},{"key":"ref25","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.170811"},{"key":"ref26","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2018.11.005"},{"key":"ref27","unstructured":"Hess DC, Sethi K, Awad E. Thrombotic thrombocytopenic purpura in systemic lupus erythematosus and antiphospholipid antibodies: effective treatment with plasma exchange and immunosuppression. J Rheumatol. 1992;19:1474-8. PMid: 1433020."},{"key":"ref28","unstructured":"Durand JM, Lefevre P, Kaplanski G, Soubeyrand J. Thrombotic microangiopathy and the antiphospholipid antibody syndrome. J Rheumatol. 1991;18:1916-8. PMid: 1795331."},{"key":"ref29","doi-asserted-by":"publisher","DOI":"10.14503\/THIJ-17-6472"},{"key":"ref30","doi-asserted-by":"publisher","DOI":"10.1038\/nrneph.2014.38"},{"key":"ref31","doi-asserted-by":"publisher","DOI":"10.1007\/s13730-018-0354-x"},{"key":"ref32","doi-asserted-by":"publisher","DOI":"10.1177\/0961203316682098"},{"key":"ref33","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2003.007245"},{"key":"ref34","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2006.067033"},{"key":"ref35","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.01753.x"},{"key":"ref36","doi-asserted-by":"publisher","DOI":"10.1002\/art.10187"},{"key":"ref37","unstructured":"\u0420\u0435\u0448\u0435\u0442\u043d\u044f\u043a \u0422.\u041c., \u0410\u043b\u0435\u043a\u0431\u0435\u0440\u043e\u0432\u0430 \u0417.\u0421., \u0410\u043b\u0435\u043a\u0441\u0430\u043d\u0434\u0440\u043e\u0432\u0430 \u0415.\u041d. \u0438 \u0434\u0440. \u041a\u043b\u0438\u043d\u0438\u0447\u0435\u0441\u043a\u0438\u0435 \u0430\u0441\u043f\u0435\u043a\u0442\u044b \u0430\u043d\u0442\u0438\u0444\u043e\u0441\u0444\u043e\u043b\u0438\u043f\u0438\u0434\u043d\u043e\u0433\u043e \u0441\u0438\u043d\u0434\u0440\u043e\u043c\u0430. \u0412\u0435\u0441\u0442\u043d. \u0420\u0410\u041c\u041d. 2003;7:31-4 [Reshetnyak TM, Alekberova ZS, Alexandrova EN, et al. Clinic-clinical aspects of antiphospholipid syndrome. Vestn. RAMS. 2003;7:31-4 (In Russ.)]."},{"key":"ref38","unstructured":"\u041d\u0430\u0441\u043e\u043d\u043e\u0432 \u0415.\u041b. \u0410\u043d\u0442\u0438\u0444\u043e\u0441\u0444\u043e\u043b\u0438\u043f\u0438\u0434\u043d\u044b\u0439 \u0441\u0438\u043d\u0434\u0440\u043e\u043c. \u041c.: \u041b\u0438\u0442\u0442\u0435\u0440\u0440\u0430, 2004 [Nasonov EL. Antifosfolipidnyi sindrom. Moscow: Litterra, 2004 (In Russ.)]."},{"key":"ref39","doi-asserted-by":"crossref","unstructured":"Garcia D, Erkan D. Diagnosis and Management of the Antiphospholipid Syndrome. N Engl J Med. 2018;378(21):2010-21. doi: 10.1056\/ NEJMra1705454","DOI":"10.1056\/NEJMra1705454"},{"key":"ref40","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2018019596"},{"key":"ref41","doi-asserted-by":"publisher","DOI":"10.1080\/0886022X.2019.1702057"},{"key":"ref42","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.0228-17"},{"key":"ref43","doi-asserted-by":"publisher","DOI":"10.1038\/nrneph.2017.85"},{"key":"ref44","doi-asserted-by":"publisher","DOI":"10.1186\/ar4142"},{"key":"ref45","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2012.05.005"},{"key":"ref46","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2018.05.007"},{"key":"ref47","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2019-215213"},{"key":"ref48","doi-asserted-by":"publisher","DOI":"10.14412\/1995-4484-2019-3-40"},{"key":"ref49","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2013.05.004"},{"key":"ref50","doi-asserted-by":"publisher","DOI":"10.1177\/1759720X14554793"},{"key":"ref51","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2017.03.012"},{"key":"ref52","doi-asserted-by":"publisher","DOI":"10.1177\/0961203318768890"},{"key":"ref53","doi-asserted-by":"crossref","unstructured":"Bhat SP, Rahman SZ. Rituximab treatment in new and refractory thrombotic thrombocytopenic purpura (TTP) patients: a review. IJMSCR. 2016;2:1-6.","DOI":"10.21276\/ijmrp.2016.2.4.001"},{"key":"ref54","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances"},{"key":"ref55","doi-asserted-by":"publisher","DOI":"10.1002\/rth2.12160"},{"key":"ref56","doi-asserted-by":"publisher","DOI":"10.1007\/s40262-019-00742-8"},{"key":"ref57","doi-asserted-by":"publisher","DOI":"10.3904\/kjim.201"},{"key":"ref58","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2019.11"},{"key":"ref59","doi-asserted-by":"publisher","DOI":"10.1007\/s11940-020-0616-8"},{"key":"ref60","doi-asserted-by":"publisher","DOI":"10.1007\/s10238-019-00565-8"},{"key":"ref61","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-017-3823-2"},{"key":"ref62","doi-asserted-by":"crossref","unstructured":"Raufi AG, Scott S, Darwish O, et al. Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab. Hematol Rep. 2016;8(3):6625. eCollection 2016 Sep 28.","DOI":"10.4081\/hr.2016.6625"},{"key":"ref63","doi-asserted-by":"publisher","DOI":"10.1053\/j.ajkd.2014.07.031"},{"key":"ref64","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kev307"},{"key":"ref65","doi-asserted-by":"publisher","DOI":"10.1002\/art.34440"},{"key":"ref66","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2011.05.007"},{"key":"ref67","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2018017095"},{"key":"ref68","doi-asserted-by":"publisher","DOI":"10.1155\/2014\/704371"},{"key":"ref69","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kev371"},{"key":"ref70","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000006338"},{"key":"ref71","doi-asserted-by":"publisher","DOI":"10.1111\/jth.13257"},{"key":"ref72","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000000143"},{"key":"ref73","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kew040"},{"key":"ref74","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/key2"},{"key":"ref75","doi-asserted-by":"publisher","DOI":"10.1016\/j.transci.2017.02.007"},{"key":"ref76","doi-asserted-by":"publisher","DOI":"10.1097\/RHU.0000000000000290"},{"key":"ref77","doi-asserted-by":"publisher","DOI":"10.1111\/ajt.12540"},{"key":"ref78","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc0910965"},{"key":"ref79","doi-asserted-by":"publisher","DOI":"10.1097\/TP.0000000000000267"},{"key":"ref80","doi-asserted-by":"publisher","DOI":"10.1111\/ajt.12319"},{"key":"ref81","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-6143.2011.03696.x"},{"key":"ref82","doi-asserted-by":"publisher","DOI":"10.1159\/000492033"},{"key":"ref83","doi-asserted-by":"publisher","DOI":"10.1016\/j.molimm.2019.07.030"},{"key":"ref84","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.00672"},{"key":"ref85","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0164646"},{"key":"ref86","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2016111179"},{"key":"ref87","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-04-420943"},{"key":"ref88","doi-asserted-by":"publisher","DOI":"10.1177\/2040620720902904"}],"container-title":["Terapevticheskii arkhiv"],"original-title":[],"link":[{"URL":"https:\/\/ter-arkhiv.ru\/0040-3660\/article\/viewFile\/34518\/pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/ter-arkhiv.ru\/0040-3660\/article\/viewFile\/34518\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,10,13]],"date-time":"2021-10-13T20:41:59Z","timestamp":1634157719000},"score":1,"resource":{"primary":{"URL":"https:\/\/ter-arkhiv.ru\/0040-3660\/article\/view\/34518"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6,5]]},"references-count":88,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2020,6,5]]}},"URL":"https:\/\/doi.org\/10.26442\/00403660.2020.05.000697","relation":{},"ISSN":["2309-5342","0040-3660"],"issn-type":[{"value":"2309-5342","type":"electronic"},{"value":"0040-3660","type":"print"}],"subject":[],"published":{"date-parts":[[2020,6,5]]}}}