{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,19]],"date-time":"2026-07-19T22:17:48Z","timestamp":1784499468530,"version":"3.55.0"},"reference-count":49,"publisher":"Springer Science and Business Media LLC","issue":"7","license":[{"start":{"date-parts":[[2022,2,18]],"date-time":"2022-02-18T00:00:00Z","timestamp":1645142400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2022,2,18]],"date-time":"2022-02-18T00:00:00Z","timestamp":1645142400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Clin Rheumatol"],"published-print":{"date-parts":[[2022,7]]},"DOI":"10.1007\/s10067-022-06055-9","type":"journal-article","created":{"date-parts":[[2022,2,18]],"date-time":"2022-02-18T05:02:42Z","timestamp":1645160562000},"page":"2021-2033","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: a study of 20 Moroccan adult patients"],"prefix":"10.1007","volume":"41","author":[{"given":"Ammouri","family":"Wafa","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harmouche","family":"Hicham","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Radi","family":"Naoufal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Khibri","family":"Hajar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Razine","family":"Rachid","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benkirane","family":"Souad","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maamar","family":"Mouna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mezalek Tazi","family":"Zoubida","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adnaoui","family":"Mohamed","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,2,18]]},"reference":[{"issue":"11","key":"6055_CR1","doi-asserted-by":"publisher","first-page":"3160","DOI":"10.1002\/art.38802","volume":"66","author":"F Minoia","year":"2014","unstructured":"Minoia F, Davi S, Horne A, Demirkaya E, Bovis F, Li C et al (2014) Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol 66(11):3160\u20133169. https:\/\/doi.org\/10.1002\/art.38802","journal-title":"Arthritis Rheumatol"},{"issue":"9","key":"6055_CR2","doi-asserted-by":"publisher","first-page":"2892","DOI":"10.1002\/art.23734","volume":"58","author":"K Zhang","year":"2008","unstructured":"Zhang K, Biroschak J, Glass DN, Thompson SD, Finkel T, Passo MH et al (2008) Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis is associated with MUNC13-4 polymorphisms. Arthritis Rheum 58(9):2892\u20132896. https:\/\/doi.org\/10.1002\/art.23734","journal-title":"Arthritis Rheum"},{"issue":"2","key":"6055_CR3","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1002\/pbc.21039","volume":"48","author":"JI Henter","year":"2007","unstructured":"Henter JI, Horne A, Arico M et al (2007) HLH-2004:\u00b4 diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis\u2019. Pediatr Blood Cancer 48(2):124\u2013131. https:\/\/doi.org\/10.1002\/pbc.21039","journal-title":"Pediatr Blood Cancer"},{"issue":"4","key":"6055_CR4","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1016\/j.blre.2014.03.002","volume":"28","author":"GE Janka","year":"2014","unstructured":"Janka GE, Lehmberg K (2014) Hemophagocytic syndromes\u2014an update. Blood Rev 28(4):135\u2013142. https:\/\/doi.org\/10.1016\/j.blre.2014.03.002","journal-title":"Blood Rev"},{"issue":"9927","key":"6055_CR5","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-Zeron 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":"5446","key":"6055_CR6","doi-asserted-by":"publisher","first-page":"1957","DOI":"10.1126\/science.286.5446.1957","volume":"286","author":"SE Stepp","year":"1999","unstructured":"Stepp SE, Dufourcq-Lagelouse R, LeDeist F et al (1999) Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science 286(5446):1957\u20131959. https:\/\/doi.org\/10.1126\/science.286.5446.1957","journal-title":"Science"},{"issue":"11","key":"6055_CR7","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. https:\/\/doi.org\/10.1093\/rheumatology\/ken342","journal-title":"Rheumatology (Oxford)"},{"issue":"4","key":"6055_CR8","doi-asserted-by":"publisher","first-page":"539","DOI":"10.1111\/bjh.13462","volume":"170","author":"K Lehmberg","year":"2015","unstructured":"Lehmberg K, Sprekels B, Nichols KE, Woessmann W, Muller I, Suttorp M et al (2015) Malignancy-associated haemophagocytic lymphohistiocytosis in children and adolescents. Br J Haematol 170(4):539\u2013549. https:\/\/doi.org\/10.1111\/bjh.13462","journal-title":"Br J Haematol"},{"issue":"17","key":"6055_CR9","doi-asserted-by":"publisher","first-page":"3229","DOI":"10.1002\/cncr.30826","volume":"123","author":"N Daver","year":"2017","unstructured":"Daver N, McClain K, Allen CE et al (2017) A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults. Cancer 123(17):3229\u20133240. https:\/\/doi.org\/10.1002\/cncr.30826","journal-title":"Cancer"},{"issue":"12","key":"6055_CR10","doi-asserted-by":"publisher","first-page":"814","DOI":"10.1016\/S1473-3099(07)70290-6","volume":"7","author":"NG Rouphael","year":"2007","unstructured":"Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C (2007) Infections associated with haemophagocytic syndrome. Lancet Infect Dis 7(12):814\u2013822. https:\/\/doi.org\/10.1016\/S1473-3099(07)70290-6","journal-title":"Lancet Infect Dis"},{"issue":"23","key":"6055_CR11","doi-asserted-by":"publisher","first-page":"2465","DOI":"10.1182\/blood.2018894618","volume":"133","author":"P La Ros\u00e9e","year":"2019","unstructured":"La Ros\u00e9e P, Horne A, Hines M et al (2019) Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood 133(23):2465\u20132477. https:\/\/doi.org\/10.1182\/blood.2018894618","journal-title":"Blood"},{"issue":"1","key":"6055_CR12","doi-asserted-by":"publisher","first-page":"86","DOI":"10.3899\/jrheum.110639","volume":"39","author":"JM Kim","year":"2012","unstructured":"Kim JM, Kwok SK, Ju JH, Kim HY, Park SH (2012) Reactive hemophagocytic syndrome in adult Korean patients with systemic lupus erythematosus: a case-control study and literature review. J Rheumatol 39(1):86\u201393. https:\/\/doi.org\/10.3899\/jrheum.110639","journal-title":"J Rheumatol"},{"issue":"5","key":"6055_CR13","doi-asserted-by":"publisher","first-page":"807","DOI":"10.1007\/s10067-006-0245-y","volume":"26","author":"YangCD QianJ","year":"2007","unstructured":"QianJ YangCD (2007) Hemophagocytic syndromeasone of main manifestations in untreated systemic lupus erythematosus: two case reports and literature review. Clin Rheumatol 26(5):807\u2013810. https:\/\/doi.org\/10.1007\/s10067-006-0245-y","journal-title":"Clin Rheumatol"},{"issue":"11","key":"6055_CR14","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) Lupus Working Group of the Paediatric Rheumatology European Society Macrophage activation syndrome in juvenile systemic lupus erythematosus: a multinational multicenter study of thirty-eight patients. Arthritis Rheum 60(11):3388\u20133399. https:\/\/doi.org\/10.1002\/art.24883","journal-title":"Arthritis Rheum"},{"issue":"7","key":"6055_CR15","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. Autoimmun Rev 16(7):743\u2013749. https:\/\/doi.org\/10.1016\/j.autrev.2017.05.010","journal-title":"Autoimmun Rev"},{"issue":"2","key":"6055_CR16","doi-asserted-by":"publisher","first-page":"100","DOI":"10.4081\/reumatismo.2018.1023","volume":"70","author":"F Dall'Ara","year":"2018","unstructured":"Dall\u2019Ara F, Cavazzana I, Frassi M et al (2018) Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center. Reumatismo 70(2):100\u2013105. https:\/\/doi.org\/10.4081\/reumatismo.2018.1023","journal-title":"Reumatismo"},{"issue":"9","key":"6055_CR17","doi-asserted-by":"publisher","first-page":"1725","DOI":"10.1002\/art.1780400928","volume":"40","author":"MC Hochberg","year":"1997","unstructured":"Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40(9):1725. https:\/\/doi.org\/10.1002\/art.1780400928","journal-title":"Arthritis Rheum"},{"key":"6055_CR18","doi-asserted-by":"publisher","first-page":"2677","DOI":"10.1002\/art.34473","volume":"64","author":"M Petri","year":"2012","unstructured":"Petri M, Orbai AM, Alarc\u00f3n GS et al (2012) Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 64:2677\u20132686. https:\/\/doi.org\/10.1002\/art.34473","journal-title":"Arthritis Rheum"},{"issue":"2","key":"6055_CR19","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1002\/pbc.21039","volume":"48","author":"JI Henter","year":"2007","unstructured":"Henter JI, Horne A, Arico M et al (2007) HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 48(2):124\u2013213. https:\/\/doi.org\/10.1002\/pbc.21039","journal-title":"Pediatr Blood Cancer"},{"issue":"6","key":"6055_CR20","doi-asserted-by":"publisher","first-page":"630","DOI":"10.1002\/art.1780350606","volume":"35","author":"C Bombardier","year":"1992","unstructured":"Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH (1992) Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE. Arthritis Rheum 35(6):630\u2013640. https:\/\/doi.org\/10.1002\/art.1780350606","journal-title":"Arthritis Rheum"},{"issue":"3","key":"6055_CR21","doi-asserted-by":"publisher","first-page":"169","DOI":"10.1097\/01.md.0000224708.62510.d1","volume":"85","author":"O Lambotte","year":"2006","unstructured":"Lambotte O, Khellaf M, Harmouche H et al (2006) Characteristics and long-term outcome of 15 episodes of systemic lupus erythematosus-associated hemophagocytic syndrome. Medicine (Baltimore) 85(3):169\u2013182. https:\/\/doi.org\/10.1097\/01.md.0000224708.62510.d1","journal-title":"Medicine (Baltimore)"},{"key":"6055_CR22","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 et al (2014) Development and validation of a score for the diagnosis of reactive hemophagocytic syndrome (HScore). Arthritis Rheumatol 66:2613\u20132620. https:\/\/doi.org\/10.1002\/art.38690","journal-title":"Arthritis Rheumatol"},{"issue":"1","key":"6055_CR23","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1093\/rheumatology\/key006","volume":"58","author":"SJ Carter","year":"2019","unstructured":"Carter SJ, Tattersall RS, Ramanan AV (2019) Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment. Rheumatology 58(1):5\u201317. https:\/\/doi.org\/10.1093\/rheumatology\/key006","journal-title":"Rheumatology"},{"issue":"1","key":"6055_CR24","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. https:\/\/doi.org\/10.1007\/s10067-017-3625-6","journal-title":"Clin Rheumatol"},{"key":"6055_CR25","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.critrevonc.2017.03.023","volume":"114","author":"R Machowicz","year":"2017","unstructured":"Machowicz R, Janka G, Wiktor-Jedrzejczak W (2017) Similar but not the same: Differential diagnosis of HLH and sepsis. Crit Rev Oncol Hematol 114:1\u201312. https:\/\/doi.org\/10.1016\/j.critrevonc.2017.03.023","journal-title":"Crit Rev Oncol Hematol"},{"key":"6055_CR26","doi-asserted-by":"publisher","first-page":"2297","DOI":"10.1002\/art.38672","volume":"66","author":"S Kumakura","year":"2014","unstructured":"Kumakura S, Murakawa Y (2014) Clinical characteristics and treatment outcomes of autoimmune associated hemophagocytic syndrome in adults. Arthritis Rheumatol 66:2297\u20132307. https:\/\/doi.org\/10.1002\/art.38672","journal-title":"Arthritis Rheumatol"},{"issue":"7","key":"6055_CR27","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, Son MB, Costenbader KH (2018) Arthritis and use of hydroxychloroquine associated with a decreased risk of macrophage activation syndrome among adult patients hospitalized with systemic lupus erythematosus. Lupus 27(7):1065\u20131071. https:\/\/doi.org\/10.1177\/0961203318759428","journal-title":"Lupus"},{"issue":"7","key":"6055_CR28","doi-asserted-by":"publisher","first-page":"1250","DOI":"10.1002\/1529-0131(199707)40:7<1250::AID-ART9>3.0.CO;2-A","volume":"40","author":"OK Eberhard","year":"1997","unstructured":"Eberhard OK, Haubitz M, Brunkhorst FM, Kliem V, Koch KM, Brunkhorst R (1997) Usefulness of procalcitonin for differentiation between activity of systemic autoimmune disease (systemic lupus erythematosus\/systemic antineutrophil cytoplasmic antibody-associated vasculitis) and invasive bacterial infection. Arthritis Rheum 40(7):1250\u20131256. https:\/\/doi.org\/10.1002\/1529-0131(199707)40:7%3c1250::AID-ART9%3e3.0.CO;2-A","journal-title":"Arthritis Rheum"},{"issue":"11","key":"6055_CR29","doi-asserted-by":"publisher","first-page":"1172","DOI":"10.1177\/0961203312450085","volume":"21","author":"KM Bador","year":"2012","unstructured":"Bador KM, Intan S, Hussin S, Gafor AHA (2012) Serum procalcitonin has negative predictive value for bacterial infection in active systemic lupus erythematosus. Lupus 21(11):1172\u20131177. https:\/\/doi.org\/10.1177\/0961203312450085","journal-title":"Lupus"},{"issue":"3","key":"6055_CR30","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1097\/RHU.0b013e3181772cca","volume":"14","author":"G Quintana","year":"2008","unstructured":"Quintana G, Medina YF, Rojas C, Fernandez A, Restrepo JF, Rondon F et al (2008) The use of procalcitonin determinations in evaluation of systemic lupus erythematosus. J Clin Rheumatol Pract Rep Rheum Musculoskelet Dis 14(3):138\u2013142. https:\/\/doi.org\/10.1097\/RHU.0b013e3181772cca","journal-title":"J Clin Rheumatol Pract Rep Rheum Musculoskelet Dis"},{"issue":"7","key":"6055_CR31","doi-asserted-by":"publisher","first-page":"586","DOI":"10.1016\/j.autrev.2015.02.005","volume":"14","author":"S Be\u00e7a","year":"2015","unstructured":"Be\u00e7a S, Rodr\u00edguez-Pint\u00f3 I, Alba MA, Cervera R, Espinosa G (2015) Development and validation of a risk calculator to differentiate flares from infections in systemic lupus erythematosus patients with fever. Autoimmun Rev 14(7):586\u2013593. https:\/\/doi.org\/10.1016\/j.autrev.2015.02.005","journal-title":"Autoimmun Rev"},{"key":"6055_CR32","doi-asserted-by":"publisher","first-page":"1805","DOI":"10.1172\/JCI18921","volume":"111","author":"MB Pepys","year":"2003","unstructured":"Pepys MB (2003) Hirschfield GM (2003) C-reactive protein: a critical update. J Clin Invest 111:1805\u20131812. https:\/\/doi.org\/10.1172\/JCI18921","journal-title":"J Clin Invest"},{"key":"6055_CR33","doi-asserted-by":"publisher","first-page":"689","DOI":"10.1002\/art.20198","volume":"50","author":"AA Grom","year":"2004","unstructured":"Grom AA (2004) Natural killer cell dysfunction: a common pathway in systemic-onset juvenile rheumatoid arthritis, macrophage activation syndrome, and hemophagocytic lymphohistiocytosis? Arthritis Rheum 50:689\u2013698. https:\/\/doi.org\/10.1002\/art.20198","journal-title":"Arthritis Rheum"},{"key":"6055_CR34","doi-asserted-by":"publisher","first-page":"108255","DOI":"10.1016\/j.clim.2019.108255","volume":"208","author":"M Usami","year":"2019","unstructured":"Usami M, Shimizu M, Mizuta M, Inoue N, Irabu H, Sakumura N, Nakagishi Y, Yachie A (2019) Extensive serum biomarker analysis in patients with macrophage activation syndrome associated with systemic lupus erythematosus. Clin Immunol 208:108255. https:\/\/doi.org\/10.1016\/j.clim.2019.108255","journal-title":"Clin Immunol"},{"key":"6055_CR35","doi-asserted-by":"publisher","first-page":"44","DOI":"10.3109\/03009742.2016.1167951","volume":"46","author":"ED Batu","year":"2017","unstructured":"Batu ED, Erden A, Seyho\u011flu E et al (2017) Assessment of the H Score for reactive haemophagocytic syndrome in patients with rheumatic diseases. Scand J Rheumatol 46:44\u201348. https:\/\/doi.org\/10.3109\/03009742.2016.1167951","journal-title":"Scand J Rheumatol"},{"key":"6055_CR36","doi-asserted-by":"publisher","first-page":"566","DOI":"10.1002\/art.39332","volume":"68","author":"A Ravelli","year":"2016","unstructured":"Ravelli A, Minoia F, Davi S et al (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 68:566\u2013576. https:\/\/doi.org\/10.1002\/art.39332","journal-title":"Arthritis Rheumatol"},{"key":"6055_CR37","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, Davi S et al (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. Ann Rheum Dis 75:481\u2013489. https:\/\/doi.org\/10.1136\/annrheumdis-2015-208982","journal-title":"Ann Rheum Dis"},{"issue":"2","key":"6055_CR38","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":"6055_CR39","doi-asserted-by":"publisher","DOI":"10.1097\/CCM.0000000000005361","author":"Melissa R Hines","year":"2021","unstructured":"Hines Melissa R, von Bahr Tatiana, Greenwood Gernot Beutel et al (2021) Consensus-based guidelines for the recognition, diagnosis, and management of hemophagocytic lymphohistiocytosis in critically ill children and adults. Crit Care Med. https:\/\/doi.org\/10.1097\/CCM.0000000000005361","journal-title":"Crit Care Med"},{"issue":"4","key":"6055_CR40","doi-asserted-by":"publisher","first-page":"617","DOI":"10.3109\/14397595.2014.906053","volume":"26","author":"Y Nakagishi","year":"2016","unstructured":"Nakagishi Y, Shimizu M, Kasai K, Miyoshi M, Yachie A (2016) Successful therapy of macrophage activation syndrome with dexamethasone palmitate. Mod Rheumatol 26(4):617\u2013620. https:\/\/doi.org\/10.3109\/14397595.2014.906053","journal-title":"Mod Rheumatol"},{"issue":"6","key":"6055_CR41","doi-asserted-by":"publisher","first-page":"899","DOI":"10.1007\/s10067-013-2227-1","volume":"3","author":"S Vilaiyuk","year":"2013","unstructured":"Vilaiyuk S, Sirachainan N, Wanitkun S, Pirojsakul K, Vaewpanich J (2013) Recurrent macrophage activation syndrome as the primary manifestation in systemic lupus erythematosus and the benefit of serial ferritin measurements: a case-based review. Clin Rheumatol 3(6):899\u2013904. https:\/\/doi.org\/10.1007\/s10067-013-2227-1","journal-title":"Clin Rheumatol"},{"issue":"1","key":"6055_CR42","doi-asserted-by":"publisher","first-page":"497","DOI":"10.1186\/s13256-021-03072-1","volume":"15","author":"Wesam Gouda","year":"2021","unstructured":"Gouda Wesam, Alsaqabi Faisal, Moshrif Abdelhfeez et al (2021) Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report. J Med Case Rep 15(1):497. https:\/\/doi.org\/10.1186\/s13256-021-03072-1","journal-title":"J Med Case Rep"},{"issue":"2","key":"6055_CR43","doi-asserted-by":"publisher","first-page":"281","DOI":"10.1007\/s10067-013-2451-8","volume":"33","author":"Y Ueda","year":"2014","unstructured":"Ueda Y, Yamashita H, Takahashi Y, Kaneko H, Kano T, Mimori A (2014) Refractory hemophagocytic syndrome in systemic lupus erythematosus successfully treated with intermittent intravenous cyclophosphamide: three case reports and literature review. Clin Rheumatol 33(2):281\u2013286. https:\/\/doi.org\/10.1007\/s10067-013-2451-8","journal-title":"Clin Rheumatol"},{"key":"6055_CR44","first-page":"108","volume":"19","author":"A Ravelli","year":"2001","unstructured":"Ravelli A, Viola S, De Benedetti F et al (2001) Dramatic efficacy of cyclosporine A in macrophage activation syndrome. Clin Exp Rheumatol 19:108","journal-title":"Clin Exp Rheumatol"},{"issue":"1","key":"6055_CR45","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1002\/ajh.1141","volume":"68","author":"U Emmenegger","year":"2001","unstructured":"Emmenegger U, Frey U, Reimers A, Fux C, Semela D, Cottagnoud P, Spaeth PJ, Neftel KA (2001) Hyperferritinemia as indicator for intravenous immunoglobulin treatment in reactive macrophage activation syndromes. Am J Hematol 68(1):4\u201310. https:\/\/doi.org\/10.1002\/ajh.1141","journal-title":"Am J Hematol"},{"key":"6055_CR46","doi-asserted-by":"publisher","first-page":"855","DOI":"10.3109\/14397595.2013.874740","volume":"24","author":"MW So","year":"2014","unstructured":"So MW, Koo BS, Kim YJ et al (2014) Successful rituximab treatment of refractory hemophagocytic lymphohistiocytosis and autoimmune hemolytic anemia associated with systemic lupus erythematosus. Mod Rheumatol 24:855\u2013857. https:\/\/doi.org\/10.3109\/14397595.2013.874740","journal-title":"Mod Rheumatol"},{"key":"6055_CR47","doi-asserted-by":"publisher","first-page":"1544","DOI":"10.1177\/0961203313504634","volume":"22","author":"J Bakshi","year":"2013","unstructured":"Bakshi J, Hassan S, D\u2019Cruz D et al (2013) Rituximab therapy in refractory macrophage activation syndrome secondary to systemic lupus erythematosus. Lupus 22:1544\u20131546. https:\/\/doi.org\/10.1177\/0961203313504634","journal-title":"Lupus"},{"issue":"1","key":"6055_CR48","doi-asserted-by":"publisher","first-page":"16","DOI":"10.1016\/j.autrev.2016.09.016","volume":"16","author":"P Ruscitti","year":"2017","unstructured":"Ruscitti P, Cipriani P, Ciccia F et al (2017) Prognostic factors of macrophage activation syndrome, at the time of diagnosis, in adult patients affected by autoimmune disease\u202f: Analysis of 41 cases collected in 2 rheumatologic centers. Autoimmun Rev 16(1):16\u201321. https:\/\/doi.org\/10.1016\/j.autrev.2016.09.016","journal-title":"Autoimmun Rev"},{"key":"6055_CR49","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1111\/1756-185X.13955","volume":"00","author":"Y Ke","year":"2020","unstructured":"Ke Y, Lv C, Xuan W et al (2020) Clinical analysis of macrophage activation syndrome in adult rheumatic disease: a multicenter retrospective study. Int J Rheum Dis 00:1\u20139. https:\/\/doi.org\/10.1111\/1756-185X.13955","journal-title":"Int J Rheum Dis"}],"container-title":["Clinical Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-022-06055-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10067-022-06055-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-022-06055-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T01:59:01Z","timestamp":1659059941000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10067-022-06055-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,2,18]]},"references-count":49,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2022,7]]}},"alternative-id":["6055"],"URL":"https:\/\/doi.org\/10.1007\/s10067-022-06055-9","relation":{},"ISSN":["0770-3198","1434-9949"],"issn-type":[{"value":"0770-3198","type":"print"},{"value":"1434-9949","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,2,18]]},"assertion":[{"value":"2 June 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 January 2022","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 January 2022","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 February 2022","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"}}]}}