{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T02:09:36Z","timestamp":1787018976095,"version":"build-2736575974"},"reference-count":24,"publisher":"SAGE Publications","issue":"2","license":[{"start":{"date-parts":[[2022,12,9]],"date-time":"2022-12-09T00:00:00Z","timestamp":1670544000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Lupus"],"published-print":{"date-parts":[[2023,2]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Hematologic manifestations are common in systemic lupus erythematosus (SLE), either at initial presentation or during the course of the disease, but data regarding their natural history are scarce.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To describe the characteristics, treatments, and outcomes of severe hematological manifestations in a large cohort of lupus patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Retrospective cohort study of patients in the \u201cAttikon\u201d lupus cohort who had a history of a severe hematologic manifestation, defined as autoimmune hemolytic anemia (AIHA) with hemoglobin &lt; 8\u00a0g\/dL, thrombocytopenia with platelet count &lt; 30,000\/mm\n                      <jats:sup>3<\/jats:sup>\n                      , Evans syndrome with hemoglobin &lt; 8\u00a0g\/dL, and\/or platelet count &lt; 30,000\/mm\n                      <jats:sup>3<\/jats:sup>\n                      , neutropenia with &lt; 500 neutrophils\/mm\n                      <jats:sup>3<\/jats:sup>\n                      , thrombotic microangiopathy (TMA)\/thrombotic thrombocytopenic purpura (TTP)-like syndrome, or macrophage activation syndrome (MAS). Demographic and clinical characteristics, treatments, and outcomes were recorded.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>From over 300 patients with hematologic manifestations, 41 qualified as severe (70.7% women, mean [SD] age at SLE diagnosis 42.6 [18.0] years). Hematologic manifestations preceded SLE diagnosis in 13 patients (31.7%), was concomitant to SLE diagnosis in 16 patients (39%), and occurred during the course of the disease in 12 (29.3%) patients, with a mean (SD) disease duration of 8.7 (5.5) years. Thrombocytopenia was the most common severe hematological manifestation (56.1%), followed by AIHA (17.1%) and TTP-like syndrome (12.2%). For initial treatment, all patients were treated with glucocorticoids (GC), while rituximab and cyclophosphamide were the most frequently used immunosuppressive agents. Following initial treatment, relapse occurred in 22 patients (53.7%). Compared to patients that did not relapse, those that relapsed had less often received concomitant immunosuppressive agents following treatment of initial episode ( n = 17\/23, 73.9% vs 5\/17, 29.4%, p = 0.005).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Severe hematologic disease in SLE has a high risk of relapse, which may be mitigated by the early institution of GC-sparing agents.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/09612033221144425","type":"journal-article","created":{"date-parts":[[2022,12,9]],"date-time":"2022-12-09T13:00:55Z","timestamp":1670590855000},"page":"225-230","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":9,"title":["Relapses are common in severe hematologic systemic lupus erythematosus and may be prevented by early institution of immunosuppressive agents: \u0391 real-life single-center study"],"prefix":"10.1177","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7591-9797","authenticated-orcid":false,"given":"Georgia-Savina","family":"Moysidou","sequence":"first","affiliation":[{"name":"Rheumatology and Clinical Immunology Unit, 4th Department of Internal Medicine, Attikon University Hospital, Athens, Greece"},{"name":"Faculty of Medicine, National and Kapodistrian University of Athens, Inflammation & Autoimmunity Lab, Biomedical Research Foundation of the Academy of Athens (BRFAA), Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Panagiotis","family":"Garantziotis","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, National and Kapodistrian University of Athens, Inflammation & Autoimmunity Lab, Biomedical Research Foundation of the Academy of Athens (BRFAA), Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9894-6966","authenticated-orcid":false,"given":"Dionysis","family":"Nikolopoulos","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, National and Kapodistrian University of Athens, Inflammation & Autoimmunity Lab, Biomedical Research Foundation of the Academy of Athens (BRFAA), Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pelagia","family":"Katsimbri","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology Unit, 4th Department of Internal Medicine, Attikon University Hospital, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonis","family":"Fanouriakis","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology Unit, 4th Department of Internal Medicine, Attikon University Hospital, Athens, Greece"},{"name":"1st Department of Propaedeutic Internal Medicine, \u201cLaiko\u201d General Hospital, Athens, Greece"},{"name":"Joint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dimitrios T","family":"Boumpas","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology Unit, 4th Department of Internal Medicine, Attikon University Hospital, Athens, Greece"},{"name":"Faculty of Medicine, National and Kapodistrian University of Athens, Inflammation & Autoimmunity Lab, Biomedical Research Foundation of the Academy of Athens (BRFAA), Athens, Greece"},{"name":"Joint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2022,12,9]]},"reference":[{"key":"bibr1-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1136\/lupus-2014-000078"},{"key":"bibr2-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keq269"},{"issue":"291","key":"bibr3-09612033221144425","first-page":"605","volume":"80","author":"Nossent JC","year":"1991","journal-title":"Q J Med"},{"key":"bibr4-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2020-217013"},{"key":"bibr5-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1016\/j.mcna.2016.09.007"},{"key":"bibr6-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2017.05.010"},{"key":"bibr7-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2020-218272"},{"key":"bibr8-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1177\/0961203316672926"},{"key":"bibr9-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000002818"},{"key":"bibr10-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2019-215089"},{"issue":"6","key":"bibr11-09612033221144425","first-page":"829","volume":"90","author":"Khellaf M","year":"2005","journal-title":"Haematologica"},{"key":"bibr12-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1177\/0961203320908932"},{"key":"bibr13-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780400928"},{"key":"bibr14-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1002\/art.34473"},{"key":"bibr15-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1002\/art.40930"},{"key":"bibr16-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2100596"},{"key":"bibr17-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.1973.tb00035.x"},{"key":"bibr18-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1191\/0961203306lu2266oa"},{"key":"bibr19-09612033221144425","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-112-9-674"},{"key":"bibr20-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24999"},{"key":"bibr21-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-06-789578"},{"key":"bibr22-09612033221144425","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2020.259481"},{"key":"bibr23-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2023325"},{"key":"bibr24-09612033221144425","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kew267"}],"container-title":["Lupus"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/09612033221144425","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/09612033221144425","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/09612033221144425","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T23:26:31Z","timestamp":1777677991000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/09612033221144425"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,9]]},"references-count":24,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2023,2]]}},"alternative-id":["10.1177\/09612033221144425"],"URL":"https:\/\/doi.org\/10.1177\/09612033221144425","relation":{},"ISSN":["0961-2033","1477-0962"],"issn-type":[{"value":"0961-2033","type":"print"},{"value":"1477-0962","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,12,9]]}}}