{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,9]],"date-time":"2026-06-09T05:59:23Z","timestamp":1780984763578,"version":"3.54.1"},"reference-count":29,"publisher":"Springer Science and Business Media LLC","issue":"6","license":[{"start":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T00:00:00Z","timestamp":1775606400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T00:00:00Z","timestamp":1775606400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Clin Rheumatol"],"published-print":{"date-parts":[[2026,6]]},"DOI":"10.1007\/s10067-026-07947-w","type":"journal-article","created":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T21:46:22Z","timestamp":1775684782000},"page":"3269-3280","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Macrophage activation syndrome in systemic lupus erythematosus: risk factors and outcomes"],"prefix":"10.1007","volume":"45","author":[{"given":"Hee Won","family":"Park","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Youngjae","family":"Park","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer Jooha","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji Hyeon","family":"Ju","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wan-Uk","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sung-Hwan","family":"Park","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6142-8364","authenticated-orcid":false,"given":"Seung-Ki","family":"Kwok","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,4,8]]},"reference":[{"issue":"9","key":"7947_CR1","doi-asserted-by":"publisher","first-page":"1151","DOI":"10.1136\/annrheumdis-2018-214819","volume":"78","author":"M Aringer","year":"2019","unstructured":"Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, Smolen JS, Wofsy D, Boumpas DT, Kamen DL, Jayne D, Cervera R, Costedoat-Chalumeau N, Diamond B, Gladman DD, Hahn B, Hiepe F, Jacobsen S, Khanna D, Lerstr\u00f8m K, Massarotti E, McCune J, Ruiz-Irastorza G, Sanchez-Guerrero J, Schneider M, Urowitz M, Bertsias G, Hoyer BF, Leuchten N, Tani C, Tedeschi SK, Touma Z, Schmajuk G, Anic B, Assan F, Chan TM, Clarke AE, Crow MK, Czirj\u00e1k L, Doria A, Graninger W, Halda-Kiss B, Hasni S, Izmirly PM, Jung M, Kum\u00e1novics G, Mariette X, Padjen I, Pego-Reigosa JM, Romero-Diaz J, R\u00faa-Figueroa Fern\u00e1ndez \u00cd, Seror R, Stummvoll GH, Tanaka Y, Tektonidou MG, Vasconcelos C, Vital EM, Wallace DJ, Yavuz S, Meroni PL, Fritzler MJ, Naden R, D\u00f6rner T, Johnson SR (2019) 2019 European league against rheumatism\/American college of rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis 78(9):1151\u20131159. https:\/\/doi.org\/10.1136\/annrheumdis-2018-214819","journal-title":"Ann Rheum Dis"},{"issue":"10","key":"7947_CR2","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.18822","volume":"13","author":"A Aziz","year":"2021","unstructured":"Aziz A, Castaneda EE, Ahmad N, Veerapalli H, Rockferry AG, Lankala CR, Hamid P (2021) Exploring macrophage activation syndrome secondary to Systemic Lupus Erythematosus in adults: a systematic review of the literature. Cureus 13(10):e18822. https:\/\/doi.org\/10.7759\/cureus.18822","journal-title":"Cureus"},{"issue":"4","key":"7947_CR3","doi-asserted-by":"publisher","first-page":"367","DOI":"10.1002\/art.43052","volume":"77","author":"PA Nigrovic","year":"2025","unstructured":"Nigrovic PA (2025) Macrophage activation syndrome. Arthritis Rheumatol 77(4):367\u2013379. https:\/\/doi.org\/10.1002\/art.43052","journal-title":"Arthritis Rheumatol"},{"issue":"6","key":"7947_CR4","doi-asserted-by":"publisher","first-page":"584","DOI":"10.1056\/NEJMra2314005","volume":"392","author":"JI Henter","year":"2025","unstructured":"Henter JI (2025) Hemophagocytic lymphohistiocytosis. N Engl J Med 392(6):584\u2013598. https:\/\/doi.org\/10.1056\/NEJMra2314005","journal-title":"N Engl J Med"},{"issue":"10","key":"7947_CR5","doi-asserted-by":"publisher","first-page":"1271","DOI":"10.1136\/ard-2023-224123","volume":"82","author":"B Shakoory","year":"2023","unstructured":"Shakoory B, Geerlinks A, Wilejto M, Kernan K, Hines M, Romano M, Piskin D, Ravelli A, Sinha R, Aletaha D, Allen C, Bassiri H, Behrens EM, Carcillo J, Carl L, Chatham W, Cohen JI, Cron RQ, Drewniak E, Grom AA, Henderson LA, Horne A, Jordan MB, Nichols KE, Schulert G, Vastert S, Demirkaya E, Goldbach-Mansky R, de Benedetti F, Marsh RA, Canna SW (2023) The 2022 EULAR\/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis\/macrophage activation syndrome (HLH\/MAS). Ann Rheum Dis 82(10):1271\u20131285. https:\/\/doi.org\/10.1136\/ard-2023-224123","journal-title":"Ann Rheum Dis"},{"issue":"2","key":"7947_CR6","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1002\/pbc.21039","volume":"48","author":"JI Henter","year":"2007","unstructured":"Henter JI, Horne A, Aric\u00f3 M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J, Janka G (2007) HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 48(2):124\u2013131. https:\/\/doi.org\/10.1002\/pbc.21039","journal-title":"Pediatr Blood Cancer"},{"issue":"1","key":"7947_CR7","doi-asserted-by":"publisher","first-page":"32","DOI":"10.1093\/rheumatology\/keae391","volume":"64","author":"F Baldo","year":"2025","unstructured":"Baldo F, Erkens RGA, Mizuta M, Rogani G, Lucioni F, Bracaglia C, Foell D, Gattorno M, Jelusic M, Anton J, Brogan P, Canna S, Chandrakasan S, Cron RQ, De Benedetti F, Grom A, Heshin-Bekenstein M, Horne A, Khubchandani R, Ozen S, Quartier P, Ravelli A, Shimizu M, Schulert G, Scott C, Sinha R, Ruperto N, Swart JF, Vastert S, Minoia F (2025) Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project. Rheumatology (Oxford) 64(1):32\u201344. https:\/\/doi.org\/10.1093\/rheumatology\/keae391","journal-title":"Rheumatology (Oxford)"},{"issue":"5","key":"7947_CR8","doi-asserted-by":"publisher","first-page":"259","DOI":"10.1038\/nrrheum.2015.179","volume":"12","author":"AA Grom","year":"2016","unstructured":"Grom AA, Horne A, De Benedetti F (2016) Macrophage activation syndrome in the era of biologic therapy. Nat Rev Rheumatol 12(5):259\u2013268. https:\/\/doi.org\/10.1038\/nrrheum.2015.179","journal-title":"Nat Rev Rheumatol"},{"issue":"2","key":"7947_CR9","doi-asserted-by":"publisher","first-page":"216","DOI":"10.1016\/j.semarthrit.2017.02.002","volume":"47","author":"SS Ahn","year":"2017","unstructured":"Ahn SS, Yoo BW, Jung SM, Lee SW, Park YB, Song JJ (2017) In-hospital mortality in febrile lupus patients based on 2016 EULAR\/ACR\/PRINTO classification criteria for macrophage activation syndrome. Semin Arthritis Rheum 47(2):216\u2013221. https:\/\/doi.org\/10.1016\/j.semarthrit.2017.02.002","journal-title":"Semin Arthritis Rheum"},{"key":"7947_CR10","doi-asserted-by":"publisher","first-page":"117","DOI":"10.2147\/oarrr.S151013","volume":"10","author":"B Lerkvaleekul","year":"2018","unstructured":"Lerkvaleekul B, Vilaiyuk S (2018) Macrophage activation syndrome: early diagnosis is key. Open Access Rheumatol 10:117\u2013128. https:\/\/doi.org\/10.2147\/oarrr.S151013","journal-title":"Open Access Rheumatol"},{"issue":"1","key":"7947_CR11","doi-asserted-by":"publisher","first-page":"e171","DOI":"10.1097\/rhu.0000000000001670","volume":"28","author":"AR Patel","year":"2022","unstructured":"Patel AR, Desai PV, Banskota SU, Edigin E, Manadan AM (2022) Hemophagocytic lymphohistiocytosis hospitalizations in adults and its association with rheumatologic diseases: data from nationwide inpatient sample. J Clin Rheumatol 28(1):e171\u2013e174. https:\/\/doi.org\/10.1097\/rhu.0000000000001670","journal-title":"J Clin Rheumatol"},{"issue":"8","key":"7947_CR12","doi-asserted-by":"publisher","first-page":"2091","DOI":"10.1007\/s10067-023-06594-9","volume":"42","author":"MER Noboa","year":"2023","unstructured":"Noboa MER, Lopez-Arevalo H, Patel AR, Arora S, Manadan AM (2023) Nationwide analysis of adult hospitalizations with hemophagocytic lymphohistiocytosis and systemic lupus erythematosus. Clin Rheumatol 42(8):2091\u20132095. https:\/\/doi.org\/10.1007\/s10067-023-06594-9","journal-title":"Clin Rheumatol"},{"issue":"6","key":"7947_CR13","doi-asserted-by":"publisher","first-page":"e358","DOI":"10.1016\/s2665-9913(20)30096-5","volume":"2","author":"P Mehta","year":"2020","unstructured":"Mehta P, Cron RQ, Hartwell J, Manson JJ, Tattersall RS (2020) Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndrome. Lancet Rheumatol 2(6):e358\u2013e367. https:\/\/doi.org\/10.1016\/s2665-9913(20)30096-5","journal-title":"Lancet Rheumatol"},{"issue":"9927","key":"7947_CR14","doi-asserted-by":"publisher","first-page":"1503","DOI":"10.1016\/s0140-6736(13)61048-x","volume":"383","author":"M Ramos-Casals","year":"2014","unstructured":"Ramos-Casals M, Brito-Zer\u00f3n P, L\u00f3pez-Guillermo A, Khamashta MA, Bosch X (2014) Adult haemophagocytic syndrome. Lancet 383(9927):1503\u20131516. https:\/\/doi.org\/10.1016\/s0140-6736(13)61048-x","journal-title":"Lancet"},{"issue":"12","key":"7947_CR15","doi-asserted-by":"publisher","first-page":"1614","DOI":"10.1136\/ard-2024-225851","volume":"83","author":"B Fautrel","year":"2024","unstructured":"Fautrel B, Mitrovic S, De Matteis A, Bindoli S, Ant\u00f3n J, Belot A, Bracaglia C, Constantin T, Dagna L, Di Bartolo A, Feist E, Foell D, Gattorno M, Georgin-Lavialle S, Giacomelli R, Grom AA, Jamilloux Y, Laskari K, Lazar C, Minoia F, Nigrovic PA, Oliveira Ramos F, Ozen S, Quartier P, Ruscitti P, Sag E, Savic S, Truchetet ME, Vastert SJ, Wilhelmer TC, Wouters C, Carmona L, De Benedetti F (2024) EULAR\/PReS recommendations for the diagnosis and management of Still\u2019s disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still\u2019s disease. Ann Rheum Dis 83(12):1614\u20131627. https:\/\/doi.org\/10.1136\/ard-2024-225851","journal-title":"Ann Rheum Dis"},{"issue":"11","key":"7947_CR16","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.27929","volume":"66","author":"MB Jordan","year":"2019","unstructured":"Jordan MB, Allen CE, Greenberg J, Henry M, Hermiston ML, Kumar A, Hines M, Eckstein O, Ladisch S, Nichols KE, Rodriguez-Galindo C, Wistinghausen B, McClain KL (2019) Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer 66(11):e27929. https:\/\/doi.org\/10.1002\/pbc.27929","journal-title":"Pediatr Blood Cancer"},{"issue":"6","key":"7947_CR17","doi-asserted-by":"publisher","first-page":"966","DOI":"10.19723\/j.issn.1671-167X.2023.06.003","volume":"55","author":"HH Yao","year":"2023","unstructured":"Yao HH, Yang F, Tang SM, Zhang X, He J, Jia Y (2023) Clinical characteristics and diagnostic indicators of macrophage activation syndrome in patients with systemic lupus erythematosus and adult-onset Still\u2019s disease. Beijing Da Xue Xue Bao Yi Xue Ban 55(6):966\u2013974. https:\/\/doi.org\/10.19723\/j.issn.1671-167X.2023.06.003","journal-title":"Beijing Da Xue Xue Bao Yi Xue Ban"},{"issue":"7","key":"7947_CR18","doi-asserted-by":"publisher","first-page":"402","DOI":"10.1016\/j.reumae.2022.09.003","volume":"19","author":"A Martins","year":"2023","unstructured":"Martins A, Pimenta S, Oliveira D, Martins F, Sam\u00f5es B, Costa L (2023) Severe and life-threatening onset of systemic lupus erythematosus. Reumatol Clin (Engl Ed) 19(7):402\u2013403. https:\/\/doi.org\/10.1016\/j.reumae.2022.09.003","journal-title":"Reumatol Clin (Engl Ed)"},{"key":"7947_CR19","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2022-252938","author":"D Shao","year":"2023","unstructured":"Shao D, Pena O, Sekulic M, Valdez Imbert R, Vegivinti CTR, Jim B (2023) Secondary haemophagocytic lymphohistiocytosis in a patient with new-onset systemic lupus erythematosus: the challenges of timely diagnosis and successful treatment. BMJ Case Rep. https:\/\/doi.org\/10.1136\/bcr-2022-252938","journal-title":"BMJ Case Rep"},{"issue":"5","key":"7947_CR20","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.60567","volume":"16","author":"AH Abdelfattah","year":"2024","unstructured":"Abdelfattah AH, Macpherson A, Javed F (2024) Macrophage activation syndrome presents as initial manifestation of lupus in an adult female. Cureus 16(5):e60567. https:\/\/doi.org\/10.7759\/cureus.60567","journal-title":"Cureus"},{"issue":"6","key":"7947_CR21","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.63167","volume":"16","author":"N Shaykh","year":"2024","unstructured":"Shaykh N, Rai O, Karan A, Bhullar KK, Tripathi V, Reddy P (2024) Unleashing the cytokine storm: a case of macrophage activation syndrome in systemic lupus erythematosus. Cureus 16(6):e63167. https:\/\/doi.org\/10.7759\/cureus.63167","journal-title":"Cureus"},{"issue":"5","key":"7947_CR22","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0267715","volume":"17","author":"SH Nam","year":"2022","unstructured":"Nam SH, Ahn SM, Oh JS, Hong S, Lee CK, Yoo B, Kim YG (2022) Macrophage activation syndrome in rheumatic disease: clinical characteristics and prognosis of 20 adult patients. PLoS ONE 17(5):e0267715. https:\/\/doi.org\/10.1371\/journal.pone.0267715","journal-title":"PLoS ONE"},{"issue":"8","key":"7947_CR23","doi-asserted-by":"publisher","DOI":"10.1002\/iid3.1364","volume":"12","author":"J Wang","year":"2024","unstructured":"Wang J, Rong W, Yan H (2024) Eighty-six cases of clinical characteristics and outcomes of systemic lupus erythematosus-associated macrophage activation syndrome: a meta-analysis study. Immun Inflamm Dis 12(8):e1364. https:\/\/doi.org\/10.1002\/iid3.1364","journal-title":"Immun Inflamm Dis"},{"issue":"3","key":"7947_CR24","doi-asserted-by":"publisher","first-page":"566","DOI":"10.1002\/art.39332","volume":"68","author":"A Ravelli","year":"2016","unstructured":"Ravelli A, Minoia F, Dav\u00ec S, Horne A, Bovis F, Pistorio A, Aric\u00f2 M, Avcin T, Behrens EM, De Benedetti F, Filipovic L, Grom AA, Henter JI, Ilowite NT, Jordan MB, Khubchandani R, Kitoh T, Lehmberg K, Lovell DJ, Miettunen P, Nichols KE, Ozen S, Pachlopnik Schmid J, Ramanan AV, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner HI, Martini A, Ruperto N, Cron RQ (2016) 2016 classification criteria for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a European league against rheumatism\/American college of rheumatology\/paediatric rheumatology international trials organisation collaborative initiative. Arthritis Rheumatol 68(3):566\u2013576. https:\/\/doi.org\/10.1002\/art.39332","journal-title":"Arthritis Rheumatol"},{"issue":"9","key":"7947_CR25","doi-asserted-by":"publisher","first-page":"2613","DOI":"10.1002\/art.38690","volume":"66","author":"L Fardet","year":"2014","unstructured":"Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D, Coppo P, Hejblum G (2014) Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol 66(9):2613\u20132620. https:\/\/doi.org\/10.1002\/art.38690","journal-title":"Arthritis Rheumatol"},{"issue":"10","key":"7947_CR26","doi-asserted-by":"publisher","first-page":"1635","DOI":"10.3324\/haematol.2017.186627","volume":"103","author":"E Gars","year":"2018","unstructured":"Gars E, Purington N, Scott G, Chisholm K, Gratzinger D, Martin BA, Ohgami RS (2018) Bone marrow histomorphological criteria can accurately diagnose hemophagocytic lymphohistiocytosis. Haematologica 103(10):1635\u20131641. https:\/\/doi.org\/10.3324\/haematol.2017.186627","journal-title":"Haematologica"},{"issue":"4","key":"7947_CR27","doi-asserted-by":"publisher","first-page":"308","DOI":"10.3899\/jrheum.2024-0910","volume":"52","author":"M Aflaki","year":"2025","unstructured":"Aflaki M, Bargman JM (2025) Urine protein tests in systemic lupus erythematosus: what do they mean? J Rheumatol 52(4):308\u2013315. https:\/\/doi.org\/10.3899\/jrheum.2024-0910","journal-title":"J Rheumatol"},{"issue":"7","key":"7947_CR28","doi-asserted-by":"publisher","first-page":"996","DOI":"10.3899\/jrheum.161286","volume":"44","author":"SS Ahn","year":"2017","unstructured":"Ahn SS, Yoo BW, Jung SM, Lee SW, Park YB, Song JJ (2017) Application of the 2016 EULAR\/ACR\/PRINTO classification criteria for macrophage activation syndrome in patients with adult-onset still disease. J Rheumatol 44(7):996\u20131003. https:\/\/doi.org\/10.3899\/jrheum.161286","journal-title":"J Rheumatol"},{"issue":"1","key":"7947_CR29","doi-asserted-by":"publisher","DOI":"10.1186\/s13054-020-02941-3","volume":"24","author":"C Knaak","year":"2020","unstructured":"Knaak C, Nyvlt P, Schuster FS, Spies C, Heeren P, Schenk T, Balzer F, La Ros\u00e9e P, Janka G, Brunkhorst FM, Keh D, Lachmann G (2020) Hemophagocytic lymphohistiocytosis in critically ill patients: diagnostic reliability of HLH-2004 criteria and Hscore. Crit Care 24(1):244. https:\/\/doi.org\/10.1186\/s13054-020-02941-3","journal-title":"Crit Care"}],"container-title":["Clinical Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-026-07947-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10067-026-07947-w","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-026-07947-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,9]],"date-time":"2026-06-09T05:25:44Z","timestamp":1780982744000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10067-026-07947-w"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,8]]},"references-count":29,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2026,6]]}},"alternative-id":["7947"],"URL":"https:\/\/doi.org\/10.1007\/s10067-026-07947-w","relation":{},"ISSN":["0770-3198","1434-9949"],"issn-type":[{"value":"0770-3198","type":"print"},{"value":"1434-9949","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,8]]},"assertion":[{"value":"28 August 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"24 December 2025","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 January 2026","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 April 2026","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"None.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Disclosures"}}]}}