{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T21:01:15Z","timestamp":1790283675755,"version":"4.1.0"},"reference-count":37,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T00:00:00Z","timestamp":1773360000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T00:00:00Z","timestamp":1773360000000},"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":[[2026,5]]},"DOI":"10.1007\/s10067-026-08010-4","type":"journal-article","created":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T09:10:19Z","timestamp":1773393019000},"page":"2665-2674","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Clinical characteristics and diagnostic indicators of childhood-onset systemic lupus erythematosus complicated with macrophage activation syndrome"],"prefix":"10.1007","volume":"45","author":[{"given":"Xin","family":"Chen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junchen","family":"Fang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peitong","family":"Han","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoyuan","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanan","family":"Han","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jingxia","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-9590-070X","authenticated-orcid":false,"given":"Chunzhen","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,3,13]]},"reference":[{"issue":"1","key":"8010_CR1","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1080\/25785826.2019.1628466","volume":"42","author":"T Sawada","year":"2019","unstructured":"Sawada T, Fujimori D, Yamamoto Y (2019) Systemic lupus erythematosus and immunodeficiency. Immunol Med 42(1):1\u20139","journal-title":"Immunol Med"},{"issue":"1","key":"8010_CR2","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1093\/rheumatology\/kead678","volume":"64","author":"Y Qian","year":"2025","unstructured":"Qian Y, Lu M, Zheng Q (2025) IL-10 and IFN-\u03b3 as markers for early recognition of pediatric systemic lupus erythematosus complicated with macrophage activation syndrome. Rheumatology (Oxford) 64(1):235\u2013241","journal-title":"Rheumatology (Oxford)"},{"issue":"4","key":"8010_CR3","doi-asserted-by":"publisher","first-page":"616","DOI":"10.1002\/art.40417","volume":"70","author":"RE Borgia","year":"2018","unstructured":"Borgia RE, Gerstein M, Levy DM et al (2018) Features, treatment, and outcomes of macrophage activation syndrome in childhood-onset systemic lupus erythematosus. Arthritis Rheum 70(4):616\u2013624","journal-title":"Arthritis Rheum"},{"issue":"11","key":"8010_CR4","doi-asserted-by":"publisher","first-page":"1686","DOI":"10.1093\/rheumatology\/ken342","volume":"47","author":"S Fukaya","year":"2008","unstructured":"Fukaya S, Yasuda S, Hashimoto T et al (2008) Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases. Rheumatology (Oxford) 47(11):1686\u20131691","journal-title":"Rheumatology (Oxford)"},{"issue":"6","key":"8010_CR5","first-page":"966","volume":"55","author":"HH Yao","year":"2023","unstructured":"Yao HH, Yang F, Tang SM et al (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","journal-title":"Beijing Da Xue Xue Bao Yi Xue Ban"},{"issue":"3","key":"8010_CR6","doi-asserted-by":"publisher","first-page":"481","DOI":"10.1136\/annrheumdis-2015-208982","volume":"75","author":"A Ravelli","year":"2016","unstructured":"Ravelli A, Minoia F, Dav\u00ec S et al (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 [J]. Ann Rheum Dis 75(3):481\u2013489","journal-title":"Ann Rheum Dis"},{"issue":"7","key":"8010_CR7","first-page":"493","volume":"51","author":"X Chen","year":"2024","unstructured":"Chen X, Fang JC, Guo JX et al (2024) A comparative study of the clinical characteristics of macrophage activation syndrome in systemic juvenile idiopathic arthritis versus systemic lupus erythematosus in children. Int J Pediatr 51(7):493\u2013498","journal-title":"Int J Pediatr"},{"issue":"11","key":"8010_CR8","doi-asserted-by":"publisher","first-page":"3388","DOI":"10.1002\/art.24883","volume":"60","author":"A Parodi","year":"2009","unstructured":"Parodi A, Dav\u00ec S, Pringe AB et al (2009) Macrophage activation syndrome in juvenile systemic lupus erythematosus: a multinational multicenter study of thirty-eight patients [J]. Arthritis Rheum 60(11):3388\u20133399","journal-title":"Arthritis Rheum"},{"issue":"2","key":"8010_CR9","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 et al (2007) HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis [J]. Pediatr Blood Cancer 48(2):124\u2013131","journal-title":"Pediatr Blood Cancer"},{"key":"8010_CR10","unstructured":"An YF, Guan FJ, Hao S et al (2020) Expert consensus on diagnosis and treatment of macrophage activation syndrome associated with rheumatic diseases in children part III: systemic lupus erythematosus in children. Chin J Pract Pediatr 35(11):835\u2013838"},{"issue":"9","key":"8010_CR11","doi-asserted-by":"publisher","first-page":"2989","DOI":"10.1002\/art.22048","volume":"54","author":"R Guti\u00e9rrez-Su\u00e1rez","year":"2006","unstructured":"Guti\u00e9rrez-Su\u00e1rez R, Ruperto N, Gastaldi R et al (2006) A proposal for a pediatric version of the Systemic Lupus International Collaborating Clinics\/American College of Rheumatology Damage Index based on the analysis of 1,015 patients with juvenile-onset systemic lupus erythematosus [J]. Arthritis Rheum 54(9):2989\u20132996","journal-title":"Arthritis Rheum"},{"issue":"1","key":"8010_CR12","doi-asserted-by":"publisher","DOI":"10.1186\/s12969-016-0130-4","volume":"15","author":"C Bracaglia","year":"2017","unstructured":"Bracaglia C, Prencipe G, De Benedetti F (2017) Macrophage activation syndrome: different mechanisms leading to a one clinical syndrome. Pediatr Rheumatol Online J 15(1):5","journal-title":"Pediatr Rheumatol Online J"},{"issue":"10","key":"8010_CR13","doi-asserted-by":"publisher","DOI":"10.1371\/journal.ppat.1002328","volume":"7","author":"M Larsen","year":"2011","unstructured":"Larsen M, Sauce D, Deback C et al (2011) Exhausted cytotoxic control of Epstein-Barr virus in human lupus. PLoS Pathog 7(10):e1002328","journal-title":"PLoS Pathog"},{"issue":"8","key":"8010_CR14","doi-asserted-by":"publisher","first-page":"487","DOI":"10.1016\/j.revmed.2022.03.341","volume":"43","author":"A Enfrein","year":"2022","unstructured":"Enfrein A, Hamidou M (2022) Epstein-Barr Virus et lupus syst\u00e9mique\u202f: quels liens\u202f? [Epstein-Barr Virus and systemic lupus: which connections?]. Rev Med Interne 43(8):487\u2013493","journal-title":"Rev Med Interne"},{"issue":"2","key":"8010_CR15","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 et al (2007) HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer 48(2):124\u2013131","journal-title":"Pediatr Blood Cancer"},{"issue":"7","key":"8010_CR16","doi-asserted-by":"publisher","first-page":"743","DOI":"10.1016\/j.autrev.2017.05.010","volume":"16","author":"PE Gavand","year":"2017","unstructured":"Gavand PE, Serio I, Arnaud L et al (2017) Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: a study of 103 episodes in 89 adult patients [J]. Autoimmun Rev 16(7):743\u2013749","journal-title":"Autoimmun Rev"},{"issue":"7","key":"8010_CR17","doi-asserted-by":"publisher","first-page":"1065","DOI":"10.1177\/0961203318759428","volume":"27","author":"EM Cohen","year":"2018","unstructured":"Cohen EM, D\u2019Silva K, Kreps D et al (2018) Arthritis and use of hydroxychloroquine associated with a decreased risk of macrophage activation syndrome among adult patients hospitalized with systemic lupus erythematosus [J]. Lupus 27(7):1065\u20131071","journal-title":"Lupus"},{"issue":"10","key":"8010_CR18","volume":"13","author":"A Aziz","year":"2021","unstructured":"Aziz A, Castaneda EE, Ahmad N et al (2021) Exploring macrophage activation syndrome secondary to systemic lupus erythematosus in adults: a systematic review of the literature [J]. Cureus 13(10):e18822","journal-title":"Cureus"},{"issue":"4","key":"8010_CR19","doi-asserted-by":"publisher","first-page":"434","DOI":"10.1016\/j.pathol.2021.07.015","volume":"54","author":"C Wilson","year":"2022","unstructured":"Wilson C, Lee WI, Cook MC et al (2022) Correlation of haemophagocytosis with clinical criteria of haemophagocytic lymphohistiocytosis and recommendations for bone marrow reporting [J]. Pathology 54(4):434\u2013441","journal-title":"Pathology"},{"issue":"3","key":"8010_CR20","volume":"15","author":"S Benlamkaddem","year":"2023","unstructured":"Benlamkaddem S, Doughmi D, Tlam\u00e7ani I et al (2023) The challenging aspect of macrophage activation syndrome in the setting of sepsis or systemic inflammatory response syndrome (SIRS) [J]. Cureus 15(3):e36228","journal-title":"Cureus"},{"issue":"9","key":"8010_CR21","doi-asserted-by":"publisher","first-page":"1450","DOI":"10.3899\/jrheum.200292","volume":"48","author":"M Gerstein","year":"2021","unstructured":"Gerstein M, Borgia RE, Dominguez D et al (2021) Predicting macrophage activation syndrome in childhood-onset systemic lupus erythematosus patients at diagnosis [J]. J Rheumatol 48(9):1450\u20131457","journal-title":"J Rheumatol"},{"issue":"1","key":"8010_CR22","doi-asserted-by":"publisher","DOI":"10.1093\/rap\/rkz013","volume":"3","author":"S Sato","year":"2019","unstructured":"Sato S, Uejima Y, Arakawa Y et al (2019) Clinical features of macrophage activation syndrome as the onset manifestation of juvenile systemic lupus erythematosus. Rheumatol Adv Pract 3(1):rkz013","journal-title":"Rheumatol Adv Pract"},{"issue":"1","key":"8010_CR23","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1007\/s10067-017-3625-6","volume":"37","author":"AC Liu","year":"2018","unstructured":"Liu AC, Yang Y, Li MT et al (2018) Macrophage activation syndrome in systemic lupus erythematosus: a multicenter, case-control study in China. Clin Rheumatol 37(1):93\u2013100","journal-title":"Clin Rheumatol"},{"issue":"4","key":"8010_CR24","doi-asserted-by":"publisher","first-page":"289","DOI":"10.1038\/gene.2012.3","volume":"13","author":"A Ravelli","year":"2012","unstructured":"Ravelli A, Grom AA, Behrens EM et al (2012) Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment. Genes Immun 13(4):289\u201398","journal-title":"Genes Immun"},{"issue":"11","key":"8010_CR25","doi-asserted-by":"publisher","first-page":"3160","DOI":"10.1002\/art.38802","volume":"66","author":"F Minoia","year":"2014","unstructured":"Minoia F, Dav\u00ec S, Horne A et al (2014) Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients [J]. Arthritis Rheumatol 66(11):3160\u20133169","journal-title":"Arthritis Rheumatol"},{"issue":"9","key":"8010_CR26","doi-asserted-by":"publisher","first-page":"1355","DOI":"10.3899\/jrheum.210024","volume":"48","author":"EA Smitherman","year":"2021","unstructured":"Smitherman EA, Cron RQ (2021) Hyperferritinemia wins again: defining macrophage activation syndrome in pediatric systemic lupus erythematosus. J Rheumatol 48(9):1355\u20131357","journal-title":"J Rheumatol"},{"issue":"10","key":"8010_CR27","doi-asserted-by":"publisher","first-page":"1082","DOI":"10.3899\/jrheum.220780","volume":"49","author":"GS Schulert","year":"2022","unstructured":"Schulert GS (2022) The big bad wolf: macrophage activation syndrome in childhood-onset systemic lupus erythematosus. J Rheumatol 49(10):1082\u20131084","journal-title":"J Rheumatol"},{"issue":"3","key":"8010_CR28","doi-asserted-by":"publisher","first-page":"625","DOI":"10.1007\/s00296-020-04763-6","volume":"41","author":"R Naveen","year":"2021","unstructured":"Naveen R, Jain A, Muhammed H, Gupta L, Misra DP, Lawrence A et al (2021) Macrophage activation syndrome in systemic lupus erythematosus and systemic-onset juvenile idiopathic arthritis: a retrospective study of similarities and dissimilarities. Rheumatol Int 41(3):625\u2013631","journal-title":"Rheumatol Int"},{"key":"8010_CR29","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2021.681875","volume":"8","author":"A Abdirakhmanova","year":"2021","unstructured":"Abdirakhmanova A, Sazonov V, Mukusheva Z et al (2021) Macrophage activation syndrome in pediatric systemic lupus erythematosus: a systematic review of the diagnostic aspects. Front Med 8:681875","journal-title":"Front Med"},{"key":"8010_CR30","doi-asserted-by":"publisher","first-page":"21","DOI":"10.1007\/978-3-031-59815-9_3","volume":"1448","author":"ED Silverman","year":"2024","unstructured":"Silverman ED (2024) The history of macrophage activation syndrome in autoimmune diseases. Adv Exp Med Biol 1448:21\u201331","journal-title":"Adv Exp Med Biol"},{"key":"8010_CR31","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2024.1389710","volume":"15","author":"Y Dong","year":"2024","unstructured":"Dong Y, Wang T, Wu H (2024) Heterogeneity of macrophage activation syndrome and treatment progression. Front Immunol 15:1389710","journal-title":"Front Immunol"},{"key":"8010_CR32","doi-asserted-by":"publisher","first-page":"237","DOI":"10.1080\/25785826.2021.1912893","volume":"44","author":"M Shimizu","year":"2021","unstructured":"Shimizu M (2021) Macrophage activation syndrome in systemic juvenile idiopathic arthritis. Immunol Med 44:237\u2013245","journal-title":"Immunol Med"},{"key":"8010_CR33","doi-asserted-by":"publisher","first-page":"2421","DOI":"10.1093\/rheumatology\/keaa634","volume":"60","author":"M Mizuta","year":"2021","unstructured":"Mizuta M, Shimizu M, Inoue N et al (2021) Clinical significance of interleukin-18 for the diagnosis and prediction of disease course in systemic juvenile idiopathic arthritis[J]. Rheumatol (Oxford) 60:2421\u20132426","journal-title":"Rheumatol (Oxford)"},{"key":"8010_CR34","doi-asserted-by":"publisher","first-page":"225","DOI":"10.1136\/annrheumdis-2019-216030","volume":"79","author":"PY Lee","year":"2020","unstructured":"Lee PY, Schulert GS, Canna SW et al (2020) Adenosine deaminase 2 as a biomarker of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Ann Rheum Dis 79:225\u2013231","journal-title":"Ann Rheum Dis"},{"key":"8010_CR35","doi-asserted-by":"publisher","first-page":"231","DOI":"10.1093\/rheumatology\/keaa299","volume":"60","author":"M Mizuta","year":"2021","unstructured":"Mizuta M, Shimizu M, Irabu H et al (2021) Comparison of serum cytokine profiles in macrophage activation syndrome complicating different background rheumatic diseases in children[J]. Rheumatol (Oxford) 60:231\u2013238","journal-title":"Rheumatol (Oxford)"},{"issue":"10","key":"8010_CR36","doi-asserted-by":"publisher","first-page":"1421","DOI":"10.1007\/s00296-016-3545-9","volume":"36","author":"S Ayta\u00e7","year":"2016","unstructured":"Ayta\u00e7 S, Batu ED, \u00dcnal \u015e et al (2016) Macrophage activation syndrome in children with systemic juvenile idiopathic arthritis and systemic lupus erythematosus. Rheumatol Int 36(10):1421\u20131429","journal-title":"Rheumatol Int"},{"issue":"3","key":"8010_CR37","doi-asserted-by":"publisher","first-page":"625","DOI":"10.1007\/s00296-020-04763-6","volume":"41","author":"R Naveen","year":"2021","unstructured":"Naveen R, Jain A, Muhammed H et al (2021) Macrophage activation syndrome in systemic lupus erythematosus and systemic-onset juvenile idiopathic arthritis: a retrospective study of similarities and dissimilarities. Rheumatol Int 41(3):625\u2013631","journal-title":"Rheumatol Int"}],"container-title":["Clinical Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-026-08010-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10067-026-08010-4","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-026-08010-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T03:49:50Z","timestamp":1775792990000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10067-026-08010-4"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,13]]},"references-count":37,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2026,5]]}},"alternative-id":["8010"],"URL":"https:\/\/doi.org\/10.1007\/s10067-026-08010-4","relation":{},"ISSN":["0770-3198","1434-9949"],"issn-type":[{"value":"0770-3198","type":"print"},{"value":"1434-9949","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,13]]},"assertion":[{"value":"13 June 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 January 2026","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 February 2026","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 March 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"}},{"value":"This study is approved by the Medical Research Ethics Committee of Children\u2019s Hospital of Hebei Province affiliated with Hebei Medical University (Approval #158) in compliance with the principles of the Declaration of Helsinki and the Code of Ethics of the World Medical Association. All methods were carried out in accordance with relevant guidelines and regulations. The Ethics Committee of Children\u2019s Hospital of Hebei Province waived the need for informed consent due to retrospective study.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"All authors agreed to the publication.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article\u2019s Creative Commons licence, unless indicated\u00a0otherwise in a credit line to the material. If material is not included in the article\u2019s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Open Access"}}]}}