{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T15:08:11Z","timestamp":1793372891327,"version":"4.5.0"},"reference-count":150,"publisher":"Elsevier BV","issue":"10","license":[{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,7,18]],"date-time":"2025-07-18T00:00:00Z","timestamp":1752796800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Autoimmunity Reviews"],"published-print":{"date-parts":[[2025,9]]},"DOI":"10.1016\/j.autrev.2025.103880","type":"journal-article","created":{"date-parts":[[2025,7,18]],"date-time":"2025-07-18T23:20:07Z","timestamp":1752880807000},"page":"103880","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":8,"title":["Managing the clinical heterogeneity of patients with Still's disease, from early diagnosis to timely treatment"],"prefix":"10.1016","volume":"24","author":[{"given":"Piero","family":"Ruscitti","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Cantarini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Ciccia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabrizio","family":"Conti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Dagna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florenzo","family":"Iannone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlomaurizio","family":"Montecucco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pistone","family":"Giovanni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Sfriso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Giacomelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"4","key":"10.1016\/j.autrev.2025.103880_bb0005","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1016\/j.pcl.2018.04.005","article-title":"Systemic Juvenile Idiopathic Arthritis","volume":"65","author":"Lee","year":"2018","journal-title":"Pediatr Clin N Am"},{"key":"10.1016\/j.autrev.2025.103880_bb0010","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.jaut.2018.07.018","article-title":"A comprehensive review on adult onset still\u2019s disease","volume":"93","author":"Giacomelli","year":"2018","journal-title":"J Autoimmun"},{"issue":"12","key":"10.1016\/j.autrev.2025.103880_bb0015","doi-asserted-by":"crossref","first-page":"1614","DOI":"10.1136\/ard-2024-225851","article-title":"EULAR\/PReS recommendations for the diagnosis and management of still\u2019s disease, comprising systemic juvenile idiopathic arthritis and adult-onset still\u2019s disease","volume":"83","author":"Fautrel","year":"2024","journal-title":"Ann Rheum Dis"},{"issue":"12","key":"10.1016\/j.autrev.2025.103880_bb0020","doi-asserted-by":"crossref","first-page":"1748","DOI":"10.1136\/ard-2024-225853","article-title":"Systemic juvenile idiopathic arthritis and adult-onset still\u2019s disease are the same disease: evidence from systematic reviews and meta-analyses informing the 2023 EULAR\/PReS recommendations for the diagnosis and management of still\u2019s disease","volume":"83","author":"De Matteis","year":"2024","journal-title":"Ann Rheum Dis"},{"issue":"2","key":"10.1016\/j.autrev.2025.103880_bb0025","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1038\/s41584-023-01065-6","article-title":"Recent advances and evolving concepts in still\u2019s disease","volume":"20","author":"Ruscitti","year":"2024","journal-title":"Nat Rev Rheumatol"},{"issue":"7","key":"10.1016\/j.autrev.2025.103880_bb0030","doi-asserted-by":"crossref","DOI":"10.1016\/j.autrev.2022.103114","article-title":"Expanding the spectrum of the hyperferritinemic syndrome, from pathogenic mechanisms to clinical observations, and therapeutic implications","volume":"21","author":"Ruscitti","year":"2022","journal-title":"Autoimmun Rev"},{"issue":"13","key":"10.1016\/j.autrev.2025.103880_bb0035","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1186\/s12969-015-0047-3","article-title":"Gene-expression analysis of adult-onset still\u2019s disease and systemic juvenile idiopathic arthritis is consistent with a continuum of a single disease entity","volume":"20","author":"Nirmala","year":"2015","journal-title":"Pediatr Rheumatol Online J"},{"key":"10.1016\/j.autrev.2025.103880_bb0040","doi-asserted-by":"crossref","unstructured":"Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still's disease. Nat Rev Rheumatol 2018 14(10):603\u2013618. doi:https:\/\/doi.org\/10.1038\/s41584-018-0081-x. PMID: 30218025; PMCID: PMC7097309.","DOI":"10.1038\/s41584-018-0081-x"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0045","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1080\/1744666X.2018.1465821","article-title":"Complications of adult-onset still\u2019s disease and their management","volume":"14","author":"Mitrovic","year":"2018","journal-title":"Expert Rev Clin Immunol"},{"issue":"3","key":"10.1016\/j.autrev.2025.103880_bb0050","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1038\/s41584-020-00571-1","article-title":"Immune cartography of macrophage activation syndrome in the COVID-19 era","volume":"17","author":"McGonagle","year":"2021","journal-title":"Nat Rev Rheumatol"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0055","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1038\/nrrheum.2015.179","article-title":"Macrophage activation syndrome in the era of biologic therapy","volume":"12","author":"Grom","year":"2016","journal-title":"Nat Rev Rheumatol"},{"issue":"11","key":"10.1016\/j.autrev.2025.103880_bb0060","doi-asserted-by":"crossref","first-page":"1773","DOI":"10.1002\/art.41071","article-title":"Storm warning: lung disease in systemic juvenile idiopathic arthritis","volume":"71","author":"Nigrovic","year":"2019","journal-title":"Arthritis Rheum"},{"issue":"9","key":"10.1016\/j.autrev.2025.103880_bb0065","doi-asserted-by":"crossref","first-page":"1152","DOI":"10.1136\/annrheumdis-2020-218048","article-title":"Lung involvement in macrophage activation syndrome and severe COVID-19: results from a cross-sectional study to assess clinical, laboratory and artificial intelligence-radiological differences","volume":"79","author":"Ruscitti","year":"2020","journal-title":"Ann Rheum Dis"},{"issue":"12","key":"10.1016\/j.autrev.2025.103880_bb0070","doi-asserted-by":"crossref","first-page":"1722","DOI":"10.1136\/annrheumdis-2019-216040","article-title":"Emergent high fatality lung disease in systemic juvenile arthritis","volume":"78","author":"Saper","year":"2019","journal-title":"Ann Rheum Dis"},{"issue":"11","key":"10.1016\/j.autrev.2025.103880_bb0075","doi-asserted-by":"crossref","first-page":"1943","DOI":"10.1002\/art.41073","article-title":"Systemic juvenile idiopathic arthritis-associated lung disease: characterization and risk factors","volume":"71","author":"Schulert","year":"2019","journal-title":"Arthritis Rheum"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0080","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1186\/s13075-020-02245-5","article-title":"Parenchymal lung disease in adult onset still\u2019s disease: an emergent marker of disease severity-characterisation and predictive factors from Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale (GIRRCS) cohort of patients","volume":"22","author":"Ruscitti","year":"2020","journal-title":"Arthritis Res Ther"},{"issue":"12","key":"10.1016\/j.autrev.2025.103880_bb0085","doi-asserted-by":"crossref","first-page":"1731","DOI":"10.1136\/ard-2024-225854","article-title":"Efficacy and safety of therapies for still\u2019s disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR\/PReS guidelines for the management of still\u2019s disease","volume":"83","author":"Bindoli","year":"2024","journal-title":"Ann Rheum Dis"},{"issue":"4","key":"10.1016\/j.autrev.2025.103880_bb0090","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1002\/art.42037","article-title":"2021 American College of Rheumatology Guideline for the treatment of juvenile idiopathic arthritis: therapeutic approaches for oligoarthritis, temporomandibular joint arthritis, and systemic juvenile idiopathic arthritis","volume":"74","author":"Onel","year":"2022","journal-title":"Arthritis Rheum"},{"issue":"4","key":"10.1016\/j.autrev.2025.103880_bb0095","doi-asserted-by":"crossref","first-page":"858","DOI":"10.1016\/j.semarthrit.2021.06.004","article-title":"Adult-onset still\u2019s disease in focus: clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies","volume":"51","author":"Efthimiou","year":"2021","journal-title":"Semin Arthritis Rheum"},{"issue":"1\u20132","key":"10.1016\/j.autrev.2025.103880_bb0100","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1080\/14712598.2024.2307340","article-title":"The latest advances in the use of biological DMARDs to treat still\u2019s disease","volume":"24","author":"Di Cola","year":"2024","journal-title":"Expert Opin Biol Ther"},{"issue":"11","key":"10.1016\/j.autrev.2025.103880_bb0105","doi-asserted-by":"crossref","first-page":"690","DOI":"10.21037\/atm-20-3875","article-title":"Retrospective analysis of 1,641 cases of classic fever of unknown origin","volume":"8","author":"Zhou","year":"2020","journal-title":"Ann Transl Med"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0110","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1186\/s41232-022-00204-y","article-title":"Clinical perspectives and therapeutic strategies: pediatric autoinflammatory disease-a multi-faceted approach to fever of unknown origin of childhood","volume":"42","author":"Yachie","year":"2022","journal-title":"Inflamm Regen"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0115","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1056\/NEJMra2111003","article-title":"Fever of unknown origin","volume":"386","author":"Haidar","year":"2022","journal-title":"N Engl J Med"},{"issue":"6","key":"10.1016\/j.autrev.2025.103880_bb0120","doi-asserted-by":"crossref","first-page":"1699","DOI":"10.1007\/s10067-019-04455-y","article-title":"Proposal for a simple algorithm to differentiate adult-onset still\u2019s disease with other fever of unknown origin causes: a longitudinal prospective study","volume":"38","author":"Bilgin","year":"2019","journal-title":"Clin Rheumatol"},{"key":"10.1016\/j.autrev.2025.103880_bb0125","article-title":"Development and validation of the AF score for diagnosis of adult-onset still\u2019s disease in fever of unknown origin","volume":"6","author":"Ying","year":"2022","journal-title":"J Transl Autoimmun"},{"issue":"4","key":"10.1016\/j.autrev.2025.103880_bb0130","doi-asserted-by":"crossref","first-page":"1125","DOI":"10.1007\/s10067-023-06509-8","article-title":"Proposal of a new diagnostic algorithm for adult-onset still\u2019s disease","volume":"42","author":"Daghor-Abbaci","year":"2023","journal-title":"Clin Rheumatol"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0135","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1016\/j.berh.2008.08.006","article-title":"Adult-onset still disease","volume":"22","author":"Fautrel","year":"2008","journal-title":"Best Pract Res Clin Rheumatol"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0140","doi-asserted-by":"crossref","first-page":"12455","DOI":"10.1038\/s41598-021-91613-5","article-title":"The joint involvement in adult onset still\u2019s disease is characterised by a peculiar magnetic resonance imaging and a specific transcriptomic profile","volume":"11","author":"Ruscitti","year":"2021","journal-title":"Sci Rep"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0145","doi-asserted-by":"crossref","first-page":"997","DOI":"10.1016\/j.rcl.2017.04.006","article-title":"Computed tomography and MR imaging in rheumatoid Arthritis","volume":"55","author":"Barile","year":"2017","journal-title":"Radiol Clin North Am"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0150","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1016\/j.jaad.2018.05.020","article-title":"Increased severity and epidermal alterations in persistent versus evanescent skin lesions in adult-onset still disease","volume":"79","author":"Zuelgaray","year":"2018","journal-title":"J Am Acad Dermatol"},{"issue":"6","key":"10.1016\/j.autrev.2025.103880_bb0155","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1016\/j.jaad.2021.02.005","article-title":"Risk of macrophage activation syndrome in patients with adult-onset still\u2019s disease with skin involvement: a retrospective cohort study","volume":"85","author":"Ding","year":"2021","journal-title":"J Am Acad Dermatol"},{"issue":"9","key":"10.1016\/j.autrev.2025.103880_bb0160","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1111\/j.1600-0560.2010.01570.x","article-title":"Persistent pruritic papules and plaques: a characteristic histopathologic presentation seen in a subset of patients with adult-onset and juvenile still\u2019s disease","volume":"37","author":"Fortna","year":"2010","journal-title":"J Cutan Pathol"},{"issue":"3","key":"10.1016\/j.autrev.2025.103880_bb0165","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/j.semarthrit.2012.05.003","article-title":"Evanescent and persistent pruritic eruptions of adult-onset still disease: a clinical and pathologic study of 36 patients","volume":"42","author":"Lee","year":"2012","journal-title":"Semin Arthritis Rheum"},{"issue":"17","key":"10.1016\/j.autrev.2025.103880_bb0170","doi-asserted-by":"crossref","first-page":"5012","DOI":"10.3390\/jcm11175012","article-title":"Evaluation of glycosylated ferritin in adult-onset still\u2019s disease and differential diagnoses","volume":"11","author":"Guerber","year":"2022","journal-title":"J Clin Med"},{"issue":"22","key":"10.1016\/j.autrev.2025.103880_bb0175","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1186\/1741-7015-11-185","article-title":"The hyperferritinemic syndrome: macrophage activation syndrome, still\u2019s disease, septic shock and catastrophic antiphospholipid syndrome","author":"Ros\u00e1rio","year":"2013","journal-title":"BMC Med"},{"issue":"7","key":"10.1016\/j.autrev.2025.103880_bb0180","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0235326","article-title":"Analysis of the multicentre Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale (GIRRCS) cohort","volume":"15","author":"Di Benedetto","year":"2020","journal-title":"PLoS One"},{"issue":"3","key":"10.1016\/j.autrev.2025.103880_bb0185","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/j.jbspin.2017.05.011","article-title":"New markers for adult-onset still\u2019s disease","volume":"85","author":"Mitrovic","year":"2018","journal-title":"Joint Bone Spine"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0190","doi-asserted-by":"crossref","first-page":"48","DOI":"10.4078\/jrd.2024.0095","article-title":"Associations between circulating interleukin-18 levels and adult-onset Still\u2019s disease: a meta-analysis","volume":"32","author":"Lee","year":"2025","journal-title":"J Rheum Dis"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0195","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1136\/annrheumdis-2015-209020","article-title":"Elevated circulating levels of interferon-\u03b3 and interferon-\u03b3-induced chemokines characterise patients with macrophage activation syndrome complicating systemic juvenile idiopathic arthritis","volume":"76","author":"Bracaglia","year":"2017","journal-title":"Ann Rheum Dis"},{"issue":"10","key":"10.1016\/j.autrev.2025.103880_bb0200","doi-asserted-by":"crossref","first-page":"3971","DOI":"10.1007\/s10067-021-05727-2","article-title":"Evaluating the multivisceral involvement on adult-onset still\u2019s disease to retrieve imaging-based differences in patients with and without macrophage activation syndrome: results from a single-centre observational study","volume":"40","author":"Di Cola","year":"2021","journal-title":"Clin Rheumatol"},{"issue":"10","key":"10.1016\/j.autrev.2025.103880_bb0205","doi-asserted-by":"crossref","first-page":"4844","DOI":"10.1093\/rheumatology\/keaa904","article-title":"Dissecting the clinical heterogeneity of adult-onset still\u2019s disease: results from a multi-dimensional characterization and stratification","volume":"60","author":"Berardicurti","year":"2021","journal-title":"Rheumatology (Oxford)"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0210","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1186\/s13075-021-02708-3","article-title":"Latent class analysis of 216 patients with adult-onset still\u2019s disease","volume":"24","author":"Sugiyama","year":"2022","journal-title":"Arthritis Res Ther"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0215","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1186\/s13075-021-02631-7","article-title":"Catastrophic adult-onset still\u2019s disease as a distinct life-threatening clinical subset: case-control study with dimension reduction analysis","volume":"23","author":"Wahbi","year":"2021","journal-title":"Arthritis Res Ther"},{"issue":"4","key":"10.1016\/j.autrev.2025.103880_bb0220","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2023-003419","article-title":"Derivation and validation of four patient clusters in still\u2019s disease, results from GIRRCS AOSD-study group and AIDA network still Disease Registry","volume":"9","author":"Ruscitti","year":"2023","journal-title":"RMD Open"},{"key":"10.1016\/j.autrev.2025.103880_bb0225","doi-asserted-by":"crossref","DOI":"10.1016\/j.smim.2022.101632","article-title":"The clinical heterogeneity of adult onset still\u2019s disease may underlie different pathogenic mechanisms. Implications for a personalised therapeutic management of these patients","volume":"58","author":"Ruscitti","year":"2021","journal-title":"Semin Immunol"},{"key":"10.1016\/j.autrev.2025.103880_bb0230","doi-asserted-by":"crossref","unstructured":"Ruscitti P, Di Cola I, Berardicurti O, Conforti A, Iacono D, Pantano I, Rozza G, Rossi S, De Stefano L, Balduzzi S, Vitale A, Caso F, Costa L, Prete M, Navarini L, Atzeni F, Guggino G, Perosa F, Cantarini L, Frediani B, Montecucco C, Ciccia F, Giacomelli R, Cipriani P. Impact of smoking habit on adult-onset Still's disease prognosis, findings from a multicentre observational study. Clin Rheumatol 2022;41(3):641-647. Doi:https:\/\/doi.org\/10.1007\/s10067-021-05929-8. Epub 2021 Oct 11. Erratum in: Clin Rheumatol. 2022 Nov;41(11):3597. doi:10.1007\/s10067-022-06357-y. PMID: 34636022.","DOI":"10.1007\/s10067-021-05929-8"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0235","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbspin.2023.105576","article-title":"Clinical characteristics of obese patients with adult-onset still\u2019s disease. Data from a large multicentre cohort","volume":"90","author":"Di Cola","year":"2023","journal-title":"Joint Bone Spine"},{"issue":"3","key":"10.1016\/j.autrev.2025.103880_bb0240","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1016\/j.semarthrit.2019.04.004","article-title":"Racial\/ethnic variations in morbidity and mortality in adult onset still\u2019s disease: an analysis of national dataset","volume":"49","author":"Mehta","year":"2019","journal-title":"Semin Arthritis Rheum"},{"issue":"8","key":"10.1016\/j.autrev.2025.103880_bb0245","first-page":"1517","article-title":"Adult-onset still\u2019s disease with elderly onset: results from a multicentre study","volume":"40","author":"Di Cola","year":"2022","journal-title":"Clin Exp Rheumatol"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0250","doi-asserted-by":"crossref","first-page":"21334","DOI":"10.1038\/s41598-022-25514-6","article-title":"Clinical features and serum cytokine profiles of elderly-onset adult-onset still\u2019s disease","volume":"12","author":"Yagishita","year":"2022","journal-title":"Sci Rep"},{"issue":"12","key":"10.1016\/j.autrev.2025.103880_bb0255","doi-asserted-by":"crossref","DOI":"10.1016\/j.autrev.2023.103400","article-title":"Expert consensus on the treatment of patients with adult-onset still\u2019s disease with the goal of achieving an early and long-term remission","volume":"22","author":"Giacomelli","year":"2023","journal-title":"Autoimmun Rev"},{"issue":"8","key":"10.1016\/j.autrev.2025.103880_bb0260","first-page":"1474","article-title":"A retrospective analysis of colchicine in combination with NSAIDs therapy in patients with systemic form of adult-onset still\u2019s disease with serositis","volume":"40","author":"Myachikova","year":"2022","journal-title":"Clin Exp Rheumatol"},{"issue":"15","key":"10.1016\/j.autrev.2025.103880_bb0265","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000015123","article-title":"Managing adult-onset still\u2019s disease: the effectiveness of high-dosage of corticosteroids as first-line treatment in inducing the clinical remission. Results from an observational study","volume":"98","author":"Ruscitti","year":"2019","journal-title":"Medicine (Baltimore)"},{"issue":"14","key":"10.1016\/j.autrev.2025.103880_bb0270","doi-asserted-by":"crossref","first-page":"1577","DOI":"10.1080\/14656566.2022.2126764","article-title":"Perspectives on the use of non-biological pharmacotherapy for adult-onset still\u2019s disease","volume":"23","author":"Di Cola","year":"2022","journal-title":"Expert Opin Pharmacother"},{"key":"10.1016\/j.autrev.2025.103880_bb0275","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1186\/s13075-022-02780-3","article-title":"Associated factors with poor treatment response to initial glucocorticoid therapy in patients with adult-onset still\u2019s disease","volume":"24","author":"Kondo","year":"2022","journal-title":"Arthritis Res Ther"},{"key":"10.1016\/j.autrev.2025.103880_bb0280","doi-asserted-by":"crossref","DOI":"10.1016\/j.semarthrit.2023.152244","article-title":"The administration of methotrexate in patients with still\u2019s disease, \u201creal-life\u201d findings from AIDA network still Disease Registry","volume":"62","author":"Ruscitti","year":"2023","journal-title":"Semin Arthritis Rheum"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0285","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1007\/s00296-019-04358-w","article-title":"Prescribing motivations and patients\u2019 characteristics related to the use of biologic drugs in adult-onset still\u2019s disease: analysis of a multicentre \u201creal-life\u201d cohort","volume":"40","author":"Ruscitti","year":"2020","journal-title":"Rheumatol Int"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0290","doi-asserted-by":"crossref","first-page":"442","DOI":"10.3899\/jrheum.2023-0995","article-title":"Systematic review and metaanalysis of pharmacological interventions in adult-onset still disease and the role of biologic disease-modifying antirheumatic drugs","volume":"51","author":"Ruscitti","year":"2024","journal-title":"J Rheumatol"},{"issue":"Suppl. 6","key":"10.1016\/j.autrev.2025.103880_bb0295","doi-asserted-by":"crossref","first-page":"vi9","DOI":"10.1093\/rheumatology\/kez350","article-title":"Anakinra in children and adults with still\u2019s disease","volume":"58","author":"Vastert","year":"2019","journal-title":"Rheumatology (Oxford)"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0300","doi-asserted-by":"crossref","first-page":"187","DOI":"10.55563\/clinexprheumatol\/fsq5vq","article-title":"The treatment of adult-onset still\u2019s disease with anakinra, a recombinant human IL-1 receptor antagonist: a systematic review of literature","volume":"39","author":"Giacomelli","year":"2021","journal-title":"Clin Exp Rheumatol"},{"key":"10.1016\/j.autrev.2025.103880_bb0305","doi-asserted-by":"crossref","DOI":"10.1177\/1759720X20933133","article-title":"The reduction of concomitant glucocorticoids dosage following treatment with IL-1 receptor antagonist in adult onset Still\u2019s disease. A systematic review and meta-analysis of observational studies","volume":"12","author":"Ruscitti","year":"2020","journal-title":"Ther Adv Musculoskelet Dis"},{"issue":"6","key":"10.1016\/j.autrev.2025.103880_bb0310","doi-asserted-by":"crossref","first-page":"1405","DOI":"10.1002\/art.38615","article-title":"Review: is there a window of opportunity for treatment of systemic juvenile idiopathic arthritis?","volume":"66","author":"Nigrovic","year":"2014","journal-title":"Arthritis Rheum"},{"issue":"2","key":"10.1016\/j.autrev.2025.103880_bb0315","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1002\/art.30128","article-title":"Anakinra as first-line disease-modifying therapy in systemic juvenile idiopathic arthritis: report of forty-six patients from an international multicenter series","volume":"63","author":"Nigrovic","year":"2011","journal-title":"Arthritis Rheum"},{"issue":"4","key":"10.1016\/j.autrev.2025.103880_bb0320","doi-asserted-by":"crossref","first-page":"1034","DOI":"10.1002\/art.38296","article-title":"Effectiveness of first-line treatment with recombinant interleukin-1 receptor antagonist in steroid-naive patients with new-onset systemic juvenile idiopathic arthritis: results of a prospective cohort study","volume":"66","author":"Vastert","year":"2014","journal-title":"Arthritis Rheum"},{"issue":"7","key":"10.1016\/j.autrev.2025.103880_bb0325","doi-asserted-by":"crossref","first-page":"1163","DOI":"10.1002\/art.40865","article-title":"Treatment to target using recombinant interleukin-1 receptor antagonist as first-line monotherapy in new-onset systemic juvenile idiopathic arthritis: results from a five-year follow-up study","volume":"71","author":"Ter Haar","year":"2019","journal-title":"Arthritis Rheum"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0330","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1002\/art.42656","article-title":"Recombinant Interleukin-1 receptor antagonist is an effective first-line treatment strategy in new-onset Systemic Juvenile Idiopathic arthritis, irrespective of HLA-DRB1 background and IL1RN variants","volume":"76","author":"Erkens","year":"2024","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.autrev.2025.103880_bb0335","doi-asserted-by":"crossref","DOI":"10.1177\/1759720X211059598","article-title":"Update on the therapy of adult-onset Still\u2019s disease with a focus on IL-1-inhibition: a systematic review","volume":"13","author":"Kedor","year":"2021","journal-title":"Ther Adv Musculoskelet Dis"},{"issue":"25","key":"10.1016\/j.autrev.2025.103880_bb0340","doi-asserted-by":"crossref","first-page":"2396","DOI":"10.1056\/NEJMoa1205099","article-title":"Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis","volume":"367","author":"Ruperto","year":"2012","journal-title":"N Engl J Med"},{"issue":"2","key":"10.1016\/j.autrev.2025.103880_bb0345","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1002\/art.41488","article-title":"Tapering canakinumab monotherapy in patients with systemic juvenile idiopathic arthritis in clinical remission: results from a phase IIIb\/IV open-label, randomized study","volume":"73","author":"Quartier","year":"2021","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.autrev.2025.103880_bb0350","doi-asserted-by":"crossref","first-page":"1114207","DOI":"10.3389\/fped.2023.1114207","article-title":"Efficacy and safety of canakinumab as a second line biologic after tocilizumab treatment failure in children with systemic juvenile idiopathic arthritis: A single-Centre cohort study using routinely collected health data","volume":"11","author":"Alexeeva","year":"2023","journal-title":"Front Pediatr"},{"issue":"8","key":"10.1016\/j.autrev.2025.103880_bb0355","doi-asserted-by":"crossref","first-page":"1090","DOI":"10.1136\/annrheumdis-2020-217155","article-title":"Canakinumab for treatment of adult-onset still\u2019s disease to achieve reduction of arthritic manifestation (CONSIDER): phase II, randomised, double-blind, placebo-controlled, multicentre, investigator-initiated trial","volume":"79","author":"Kedor","year":"2020","journal-title":"Ann Rheum Dis"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0360","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/j.semarthrit.2020.10.011","article-title":"Outcome of refractory to conventional and\/or biologic treatment adult Still\u2019s disease following canakinumab treatment: countrywide data in 50 patients","volume":"51","author":"Laskari","year":"2021","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.autrev.2025.103880_bb0365","doi-asserted-by":"crossref","DOI":"10.3389\/fmed.2022.1071732","article-title":"Canakinumab as first-line biological therapy in still\u2019s disease and differences between the systemic and the chronic-articular courses: real-life experience from the international AIDA registry","volume":"9","author":"Vitale","year":"2022","journal-title":"Front Med"},{"key":"10.1016\/j.autrev.2025.103880_bb0370","doi-asserted-by":"crossref","DOI":"10.3389\/fmed.2023.1256243","article-title":"Efficacy of canakinumab in patients with still\u2019s disease across different lines of biologic therapy: real-life data from the international AIDA network Registry for still\u2019s Disease","volume":"10","author":"Vitale","year":"2023","journal-title":"Front Med"},{"issue":"19","key":"10.1016\/j.autrev.2025.103880_bb0375","doi-asserted-by":"crossref","first-page":"4400","DOI":"10.3390\/jcm10194400","article-title":"Distinct effects of interleukin-1\u03b2 inhibition upon cytokine profile in patients with adult-onset still\u2019s disease and active articular manifestation responding to canakinumab","volume":"10","author":"Ghannam","year":"2021","journal-title":"J Clin Med"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0380","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1186\/s13075-016-1212-x","volume":"19","author":"Brachat","year":"2017","journal-title":"Arthritis Res Ther"},{"issue":"7","key":"10.1016\/j.autrev.2025.103880_bb0385","doi-asserted-by":"crossref","first-page":"1334","DOI":"10.1002\/art.41640","article-title":"Distinct gene expression signatures characterize strong clinical responders versus nonresponders to canakinumab in children with systemic juvenile idiopathic arthritis","volume":"73","author":"Verweyen","year":"2021","journal-title":"Arthritis Rheum"},{"issue":"4","key":"10.1016\/j.autrev.2025.103880_bb0390","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1080\/14712598.2019.1590334","article-title":"Tocilizumab for the treatment of adult-onset still\u2019s disease","volume":"19","author":"Casta\u00f1eda","year":"2019","journal-title":"Expert Opin Biol Ther"},{"issue":"25","key":"10.1016\/j.autrev.2025.103880_bb0395","doi-asserted-by":"crossref","first-page":"2385","DOI":"10.1056\/NEJMoa1112802","article-title":"Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis","volume":"367","author":"De Benedetti","year":"2012","journal-title":"N Engl J Med"},{"issue":"12","key":"10.1016\/j.autrev.2025.103880_bb0400","doi-asserted-by":"crossref","first-page":"1720","DOI":"10.1136\/annrheumdis-2018-213920","article-title":"Tocilizumab in patients with adult-onset still\u2019s disease refractory to glucocorticoid treatment: a randomised, double-blind, placebo-controlled phase III trial","volume":"77","author":"Kaneko","year":"2018","journal-title":"Ann Rheum Dis"},{"issue":"2","key":"10.1016\/j.autrev.2025.103880_bb0405","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1007\/s00296-016-3595-z","article-title":"Comparisons of the outcomes between early and late tocilizumab treatment in systemic juvenile idiopathic arthritis","volume":"37","author":"Pacharapakornpong","year":"2017","journal-title":"Rheumatol Int"},{"key":"10.1016\/j.autrev.2025.103880_bb0410","doi-asserted-by":"crossref","unstructured":"Yan X, Tang W, Zhang Z, Zhang Y, Luo C, Tang X. Tocilizumab in Systemic Juvenile Idiopathic Arthritis: response differs by Disease duration at medication initiation and by phenotype of disease. Front Pediatr 2021 9:735846. doi:https:\/\/doi.org\/10.3389\/fped.2021.735846 PMID: 34820342; PMCID: PMC8606744.","DOI":"10.3389\/fped.2021.735846"},{"key":"10.1016\/j.autrev.2025.103880_bb0415","doi-asserted-by":"crossref","DOI":"10.3389\/fmed.2021.665028","article-title":"Short-term outcomes and predictors of effectiveness of tocilizumab in systemic juvenile idiopathic arthritis: a prospective cohort study","volume":"8","author":"Nada","year":"2021","journal-title":"Front Med"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0420","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1080\/14397595.2017.1416924","article-title":"Efficacy and safety of tocilizumab with inhibition of interleukin-6 in adult-onset still\u2019s disease: a meta-analysis","volume":"28","author":"Ma","year":"2018","journal-title":"Mod Rheumatol"},{"key":"10.1016\/j.autrev.2025.103880_bb0425","doi-asserted-by":"crossref","DOI":"10.1016\/j.semarthrit.2022.152089","article-title":"Efficacy and safety of tocilizumab in adult-onset still\u2019s disease: real-life experience from the international AIDA registry","volume":"57","author":"Sota","year":"2022","journal-title":"Semin Arthritis Rheum"},{"issue":"4","key":"10.1016\/j.autrev.2025.103880_bb0430","doi-asserted-by":"crossref","first-page":"1594","DOI":"10.1093\/rheumatology\/keac440","article-title":"JAK inhibitors in difficult-to-treat adult-onset still\u2019s disease and systemic-onset juvenile idiopathic arthritis","volume":"62","author":"Gillard","year":"2023","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.autrev.2025.103880_bb0435","doi-asserted-by":"crossref","DOI":"10.1016\/j.intimp.2024.112451","article-title":"The efficacy and safety of JAK inhibitors in patients with adult-onset still\u2019s disease: a meta-analysis and systematic review","volume":"137","author":"Liao","year":"2024","journal-title":"Int Immunopharmacol"},{"issue":"10","key":"10.1016\/j.autrev.2025.103880_bb0440","doi-asserted-by":"crossref","first-page":"4124","DOI":"10.1093\/rheumatology\/keac027","article-title":"4 in the prevalence of clinical features between systemic juvenile idiopathic arthritis and adult-onset still\u2019s disease","volume":"61","author":"Ruscitti","year":"2022","journal-title":"Rheumatology (Oxford)"},{"issue":"4","key":"10.1016\/j.autrev.2025.103880_bb0445","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2023-003578","article-title":"Still\u2019s disease continuum from childhood to elderly: data from the international AIDA network still\u2019s disease registry","volume":"9","author":"Vitale","year":"2023","journal-title":"RMD Open"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0450","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1007\/s10067-013-2381-5","article-title":"Tocilizumab for the treatment of adult-onset still\u2019s disease: results from a case series","volume":"33","author":"Cipriani","year":"2014","journal-title":"Clin Rheumatol"},{"issue":"8","key":"10.1016\/j.autrev.2025.103880_bb0455","doi-asserted-by":"crossref","first-page":"369","DOI":"10.3389\/fphar.2017.00369","article-title":"Response to interleukin-1 inhibitors in 140 Italian patients with adult-onset still\u2019s disease: a multicentre retrospective observational study","author":"Colafrancesco","year":"2017","journal-title":"Front Pharmacol"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0460","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1002\/acr.20319","article-title":"Tocilizumab in refractory adult still\u2019s disease","volume":"63","author":"Pu\u00e9chal","year":"2011","journal-title":"Arthritis Care Res"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0465","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbspin.2022.105499","article-title":"Derivation and validation of adult still activity score (SAS)","volume":"90","author":"Kalyoncu","year":"2023","journal-title":"Joint Bone Spine"},{"issue":"Suppl. 9","key":"10.1016\/j.autrev.2025.103880_bb0470","article-title":"The development of the EULAR score for the definition of disease activity in adult-onset still\u2019s disease; the \u201cDAVID\u201d project [abstract]","volume":"76","author":"Ruscitti","year":"2024","journal-title":"Arthritis Rheum"},{"issue":"6","key":"10.1016\/j.autrev.2025.103880_bb0475","doi-asserted-by":"crossref","first-page":"864","DOI":"10.3899\/jrheum.170955","article-title":"Macrophage activation syndrome in patients affected by adult-onset still disease: analysis of survival rates and predictive factors in the gruppo italiano di ricerca in reumatologia clinica e sperimentale cohort","volume":"45","author":"Ruscitti","year":"2018","journal-title":"J Rheumatol"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0480","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1016\/j.autrev.2016.09.016","article-title":"Prognostic factors of macrophage activation syndrome, at the time of diagnosis, in adult patients affected by autoimmune disease: analysis of 41 cases collected in 2 rheumatologic centers","volume":"16","author":"Ruscitti","year":"2017","journal-title":"Autoimmun Rev"},{"issue":"2","key":"10.1016\/j.autrev.2025.103880_bb0485","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1097\/00005792-199103000-00004","article-title":"Adult still\u2019s disease: manifestations, disease course, and outcome in 62 patients","volume":"70","author":"Pouchot","year":"1991","journal-title":"Medicine (Baltimore)"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0490","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1186\/s12916-016-0738-8","article-title":"Adult-onset still\u2019s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers","volume":"14","author":"Ruscitti","year":"2016","journal-title":"BMC Med"},{"issue":"7","key":"10.1016\/j.autrev.2025.103880_bb0495","doi-asserted-by":"crossref","first-page":"1141","DOI":"10.1002\/art.42845","article-title":"The Systemic score may identify life-threatening evolution in still Disease: data from the GIRRCS AOSD-study group and the AIDA network still Disease Registry","volume":"76","author":"Ruscitti","year":"2024","journal-title":"Arthritis Rheum"},{"issue":"6","key":"10.1016\/j.autrev.2025.103880_bb0500","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1002\/acr2.11048","article-title":"Ferritin to erythrocyte sedimentation rate ratio: simple measure to identify macrophage activation syndrome in Systemic Juvenile Idiopathic Arthritis","volume":"1","author":"Eloseily","year":"2019","journal-title":"ACR Open Rheumatol"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0505","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1007\/s12519-019-00256-0","article-title":"Clinical and laboratory features, treatment, and outcomes of macrophage activation syndrome in 80 children: a multi-center study in China","volume":"16","author":"Zou","year":"2020","journal-title":"World J Pediatr"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0510","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1093\/rheumatology\/keac247","article-title":"Cytokine profile, ferritin and multi-visceral involvement characterize macrophage activation syndrome during adult-onset still\u2019s disease","volume":"62","author":"Ruscitti","year":"2022","journal-title":"Rheumatology (Oxford)"},{"issue":"10","key":"10.1016\/j.autrev.2025.103880_bb0515","doi-asserted-by":"crossref","first-page":"1271","DOI":"10.1136\/ard-2023-224123","article-title":"The 2022 EULAR\/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis\/macrophage activation syndrome (HLH\/MAS)","volume":"82","author":"Shakoory","year":"2023","journal-title":"Ann Rheum Dis"},{"issue":"7","key":"10.1016\/j.autrev.2025.103880_bb0520","doi-asserted-by":"crossref","first-page":"2367","DOI":"10.1182\/blood-2002-01-0172","article-title":"Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation","volume":"100","author":"Henter","year":"2002","journal-title":"Blood"},{"issue":"2","key":"10.1016\/j.autrev.2025.103880_bb0525","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1002\/pbc.21039","article-title":"HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis","volume":"48","author":"Henter","year":"2007","journal-title":"Pediatr Blood Cancer"},{"issue":"3","key":"10.1016\/j.autrev.2025.103880_bb0530","doi-asserted-by":"crossref","first-page":"566","DOI":"10.1002\/art.39332","volume":"68","author":"Ravelli","year":"2016","journal-title":"Arthritis Rheum"},{"issue":"9","key":"10.1016\/j.autrev.2025.103880_bb0535","doi-asserted-by":"crossref","first-page":"2613","DOI":"10.1002\/art.38690","article-title":"Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome","volume":"66","author":"Fardet","year":"2014","journal-title":"Arthritis Rheum"},{"issue":"10","key":"10.1016\/j.autrev.2025.103880_bb0540","doi-asserted-by":"crossref","first-page":"1357","DOI":"10.1136\/annrheumdis-2019-215211","article-title":"Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis","volume":"78","author":"Minoia","year":"2019","journal-title":"Ann Rheum Dis"},{"issue":"9927","key":"10.1016\/j.autrev.2025.103880_bb0545","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1016\/S0140-6736(13)61048-X","article-title":"Adult haemophagocytic syndrome","volume":"383","author":"Ramos-Casals","year":"2014","journal-title":"Lancet"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0550","first-page":"11","article-title":"Calcineurin inhibitors for adult-onset Still\u2019s disease: a multicentre retrospective cohort study","volume":"127","author":"Nakamura","year":"2020","journal-title":"Clin Exp Rheumatol"},{"issue":"9","key":"10.1016\/j.autrev.2025.103880_bb0555","doi-asserted-by":"crossref","first-page":"2817","DOI":"10.1007\/s10067-022-06184-1","article-title":"Short-term, low-dose etoposide in refractory adult-onset still\u2019s disease-associated macrophage activation syndrome","volume":"41","author":"Wang","year":"2022","journal-title":"Clin Rheumatol"},{"issue":"10","key":"10.1016\/j.autrev.2025.103880_bb0560","doi-asserted-by":"crossref","first-page":"1596","DOI":"10.3899\/jrheum.200941","article-title":"Efficacy of moderately dosed etoposide in macrophage activation syndrome-hemophagocytic lymphohistiocytosis","volume":"48","author":"Horne","year":"2021","journal-title":"J Rheumatol"},{"issue":"6","key":"10.1016\/j.autrev.2025.103880_bb0565","doi-asserted-by":"crossref","first-page":"1114","DOI":"10.1093\/mr\/roab119","article-title":"Effective therapy of tocilizumab on systemic juvenile idiopathic arthritis-associated refractory macrophage activation syndrome","volume":"32","author":"Wu","year":"2022","journal-title":"Mod Rheumatol"},{"issue":"6","key":"10.1016\/j.autrev.2025.103880_bb0570","doi-asserted-by":"crossref","first-page":"1164","DOI":"10.3390\/jcm10061164","article-title":"The pathogenic role of interferons in the Hyperinflammatory response on adult-onset still\u2019s disease and macrophage activation syndrome: paving the way towards new therapeutic targets","volume":"10","author":"Di Cola","year":"2021","journal-title":"J Clin Med"},{"issue":"11","key":"10.1016\/j.autrev.2025.103880_bb0575","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1038\/s41584-021-00694-z","article-title":"Targeting interferon-\u03b3 in hyperinflammation: opportunities and challenges","volume":"17","author":"De Benedetti","year":"2021","journal-title":"Nat Rev Rheumatol"},{"issue":"6","key":"10.1016\/j.autrev.2025.103880_bb0580","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1136\/ard-2022-223739","article-title":"Efficacy and safety of emapalumab in macrophage activation syndrome","volume":"82","author":"De Benedetti","year":"2023","journal-title":"Ann Rheum Dis"},{"issue":"19","key":"10.1016\/j.autrev.2025.103880_bb0585","doi-asserted-by":"crossref","first-page":"1811","DOI":"10.1056\/NEJMoa1911326","article-title":"Emapalumab in children with primary hemophagocytic lymphohistiocytosis","volume":"382","author":"Locatelli","year":"2020","journal-title":"N Engl J Med"},{"issue":"4","key":"10.1016\/j.autrev.2025.103880_bb0590","doi-asserted-by":"crossref","first-page":"761","DOI":"10.1111\/bjh.17331","article-title":"Ruxolitinib-combined doxorubicin-etoposide-methylprednisolone regimen as a salvage therapy for refractory\/relapsed haemophagocytic lymphohistiocytosis: a single-arm, multicentre, phase 2 trial","volume":"193","author":"Wang","year":"2021","journal-title":"Br J Haematol"},{"issue":"12","key":"10.1016\/j.autrev.2025.103880_bb0595","doi-asserted-by":"crossref","first-page":"1798","DOI":"10.1136\/ard-2024-226433","article-title":"Ruxolitinib as a salvage therapy in adult-onset macrophage activation syndrome: insights from eight cases","volume":"83","author":"Song","year":"2024","journal-title":"Ann Rheum Dis"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0600","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1016\/j.hoc.2015.06.011","article-title":"The role of hematopoietic stem cell transplantation in treatment of hemophagocytic lymphohistiocytosis","volume":"29","author":"Nikiforow","year":"2015","journal-title":"Hematol Oncol Clin North Am"},{"issue":"23","key":"10.1016\/j.autrev.2025.103880_bb0605","doi-asserted-by":"crossref","first-page":"2465","DOI":"10.1182\/blood.2018894618","article-title":"Recommendations for the management of hemophagocytic lymphohistiocytosis in adults","volume":"133","author":"La Ros\u00e9e","year":"2019","journal-title":"Blood"},{"issue":"1","key":"10.1016\/j.autrev.2025.103880_bb0610","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1186\/s12969-019-0309-6","article-title":"Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome","volume":"17","author":"Halyabar","year":"2019","journal-title":"Pediatr Rheumatol Online J"},{"issue":"5","key":"10.1016\/j.autrev.2025.103880_bb0615","doi-asserted-by":"crossref","first-page":"860","DOI":"10.1097\/CCM.0000000000005361","article-title":"Consensus-based guidelines for the recognition, diagnosis, and management of hemophagocytic lymphohistiocytosis in critically ill children and adults","volume":"50","author":"Hines","year":"2022","journal-title":"Crit Care Med"},{"issue":"3","key":"10.1016\/j.autrev.2025.103880_bb0620","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1007\/s10067-014-2487-4","article-title":"Life-threatening complications of adult-onset Still\u2019s disease","volume":"33","author":"Efthimiou","year":"2014","journal-title":"Clin Rheumatol"},{"key":"10.1016\/j.autrev.2025.103880_bb0625","article-title":"The evaluation of myocarditis in patients with Still\u2019s disease; clinical findings from the multicentre international AIDA Network Still\u2019s Disease Registry","author":"Ruscitti","year":"2024","journal-title":"J Rheumatol"},{"issue":"23","key":"10.1016\/j.autrev.2025.103880_bb0630","article-title":"A cohort study of liver involvement in patients With adult-onset still\u2019s Disease: prevalence, characteristics and impact on prognosis","author":"Chi","year":"2020","journal-title":"Front Med"},{"key":"10.1016\/j.autrev.2025.103880_bb0635","doi-asserted-by":"crossref","DOI":"10.1093\/rheumatology\/keaf021","article-title":"The clinical assessment of lung involvement in patients with Still\u2019s disease, results from the multicentre international AIDA Network Still\u2019s Disease Registry","author":"Ruscitti","year":"2025","journal-title":"Rheumatology (Oxford)"},{"issue":"10","key":"10.1016\/j.autrev.2025.103880_bb0640","doi-asserted-by":"crossref","first-page":"2063","DOI":"10.1002\/acr.25129","article-title":"Incidence and risk factors for eosinophilia and lung disease in biologic-exposed children with systemic juvenile idiopathic arthritis","volume":"75","author":"Wobma","year":"2023","journal-title":"Arthritis Care Res"},{"issue":"10","key":"10.1016\/j.autrev.2025.103880_bb0645","doi-asserted-by":"crossref","first-page":"2082","DOI":"10.1002\/acr.25132","article-title":"HLA-DRB1*15 and eosinophilia are common among patients with systemic juvenile idiopathic arthritis","volume":"75","author":"Lerman","year":"2023","journal-title":"Arthritis Care Res"},{"key":"10.1016\/j.autrev.2025.103880_bb0650","article-title":"Disease course, treatments, and outcomes of children with systemic juvenile idiopathic arthritis associated lung disease (SJIA-LD)","author":"Huang","year":"2023","journal-title":"Arthritis Care Res"},{"issue":"7","key":"10.1016\/j.autrev.2025.103880_bb0655","doi-asserted-by":"crossref","first-page":"1122","DOI":"10.1002\/art.42137","article-title":"The conundrum of lung disease and drug hypersensitivity-like reactions in systemic juvenile idiopathic arthritis","volume":"74","author":"Binstadt","year":"2022","journal-title":"Arthritis Rheum"},{"issue":"10","key":"10.1016\/j.autrev.2025.103880_bb0660","doi-asserted-by":"crossref","first-page":"556","DOI":"10.1002\/acr2.11600","article-title":"Development of a screening algorithm for lung disease in systemic juvenile idiopathic arthritis","volume":"5","author":"Wobma","year":"2023","journal-title":"ACR Open Rheumatol"},{"issue":"23","key":"10.1016\/j.autrev.2025.103880_bb0665","doi-asserted-by":"crossref","first-page":"2255","DOI":"10.1056\/NEJMra2026131","article-title":"Cytokine storm","volume":"383","author":"Fajgenbaum","year":"2020","journal-title":"N Engl J Med"},{"issue":"2","key":"10.1016\/j.autrev.2025.103880_bb0670","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1038\/nri3800","article-title":"Role of innate and adaptive immune mechanisms in cardiac injury and repair","volume":"15","author":"Epelman","year":"2015","journal-title":"Nat Rev Immunol"},{"issue":"17","key":"10.1016\/j.autrev.2025.103880_bb0675","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1097\/MD.0000000000000112","article-title":"Myocarditis in adult-onset still disease","volume":"93","author":"Gerfaud-Valentin","year":"2014","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.autrev.2025.103880_bb0680","doi-asserted-by":"crossref","DOI":"10.1016\/j.jaut.2020.102541","article-title":"Cardiac involvement in adult-onset still\u2019s disease: manifestations, treatments and outcomes in a retrospective study of 28 patients","volume":"116","author":"Bodard","year":"2021","journal-title":"J Autoimmun"},{"issue":"3","key":"10.1016\/j.autrev.2025.103880_bb0685","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1038\/s41569-020-00435-x","article-title":"Myocarditis and inflammatory cardiomyopathy: current evidence and future directions","volume":"18","author":"Tsch\u00f6pe","year":"2021","journal-title":"Nat Rev Cardiol"},{"key":"10.1016\/j.autrev.2025.103880_bb0690","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCHEARTFAILURE.120.007405","article-title":"Management of acute myocarditis and chronic inflammatory cardiomyopathy: an expert consensus document","volume":"13","author":"Ammirati","year":"2020","journal-title":"Circ Heart Fail"},{"issue":"2","key":"10.1016\/j.autrev.2025.103880_bb0695","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1002\/art.1780300209","article-title":"Adult-onset still\u2019s disease. Clinical course and outcome","volume":"30","author":"Cush","year":"1987","journal-title":"Arthritis Rheum"},{"issue":"11","key":"10.1016\/j.autrev.2025.103880_bb0700","doi-asserted-by":"crossref","first-page":"1041","DOI":"10.1080\/1744666X.2017.1372194","article-title":"Advances in immunopathogenesis of macrophage activation syndrome during rheumatic inflammatory diseases: toward new therapeutic targets?","volume":"13","author":"Ruscitti","year":"2017","journal-title":"Expert Rev Clin Immunol"},{"issue":"6","key":"10.1016\/j.autrev.2025.103880_bb0705","doi-asserted-by":"crossref","first-page":"2535","DOI":"10.1093\/rheumatology\/keab726","article-title":"Adult-onset still\u2019s disease or systemic-onset juvenile idiopathic arthritis and spondyloarthritis: overlapping syndrome or phenotype shift?","volume":"61","author":"Mitrovic","year":"2022","journal-title":"Rheumatology (Oxford)"},{"issue":"12","key":"10.1016\/j.autrev.2025.103880_bb0710","doi-asserted-by":"crossref","first-page":"2633","DOI":"10.3390\/jcm10122633","article-title":"Clinical phenotypes of adult-onset still\u2019s Disease: new insights from pathophysiology and literature findings","volume":"10","author":"Mitrovic","year":"2021","journal-title":"J Clin Med"},{"issue":"9","key":"10.1016\/j.autrev.2025.103880_bb0715","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1016\/j.autrev.2017.07.012","volume":"16","author":"Giacomelli","year":"2017","journal-title":"Autoimmun Rev"},{"issue":"2","key":"10.1016\/j.autrev.2025.103880_bb0720","doi-asserted-by":"crossref","DOI":"10.1016\/j.autrev.2020.102738","article-title":"The growing role of precision medicine for the treatment of autoimmune diseases; results of a systematic review of literature and Experts\u2019 Consensus","volume":"20","author":"Giacomelli","year":"2021","journal-title":"Autoimmun Rev"},{"issue":"7\u20138","key":"10.1016\/j.autrev.2025.103880_bb0725","article-title":"Tailoring the treatment of inflammatory rheumatic diseases by a better stratification and characterization of the clinical patient heterogeneity. Findings from a systematic literature review and experts\u2019 consensus","volume":"23","author":"Ruscitti","year":"2024","journal-title":"Autoimmun Rev"},{"issue":"2","key":"10.1016\/j.autrev.2025.103880_bb0730","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1097\/MD.0000000000000021","article-title":"Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients","volume":"93","author":"Gerfaud-Valentin","year":"2014","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.autrev.2025.103880_bb0735","doi-asserted-by":"crossref","unstructured":"Kalyoncu U, Solmaz D, Emmungil H, Yazici A, Kasifoglu T, Kimyon G, Balkarli A, Bes C, Ozmen M, Alibaz-Oner F, Erten S, Cagatay Y, Cetin GY, Yilmaz S, Yildiz F, Pamuk ON, Kucuksahin O, Kilic L, Yazisiz V, Karadag O, Koca SS, Hayran M, Akar S, Aksu K, Akkoc N, Keser G, Gonullu E, Kisacik B, Onat AM, Soy M, Inanc N, Direskeneli H, Sayarlioglu M, Erken E, Turgay M, Cefle A, Ertenli I, Pay S. Response rate of initial conventional treatments, disease course, and related factors of patients with adult-onset Still's disease: data from a large multicenter cohort. J Autoimmun 2016 69:59\u201363. doi:https:\/\/doi.org\/10.1016\/j.jaut.2016.02.010. Epub 2016 Mar 9. PMID: 26970681.","DOI":"10.1016\/j.jaut.2016.02.010"},{"issue":"9","key":"10.1016\/j.autrev.2025.103880_bb0740","doi-asserted-by":"crossref","first-page":"4152","DOI":"10.1093\/rheumatology\/keaa899","article-title":"Adult-onset still\u2019s disease with neurological involvement: a single-centre report","volume":"60","author":"Zhao","year":"2021","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.autrev.2025.103880_bb0745","article-title":"Epidemiology, characteristics, treatments, and outcomes of adult-onset still\u2019s disease in afro-Caribbeans: results from a population-based study in Martinique","volume":"139","author":"de Fritsch","year":"2023","journal-title":"French West Indies J Autoimmun"},{"key":"10.1016\/j.autrev.2025.103880_bb0750","article-title":"Development and implementation of the AIDA international Registry for patients With still\u2019s Disease","volume":"9","author":"Vitale","year":"2022","journal-title":"Front Med"}],"container-title":["Autoimmunity Reviews"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1568997225001405?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1568997225001405?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,12,2]],"date-time":"2025-12-02T04:11:30Z","timestamp":1764648690000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1568997225001405"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,9]]},"references-count":150,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2025,9]]}},"alternative-id":["S1568997225001405"],"URL":"https:\/\/doi.org\/10.1016\/j.autrev.2025.103880","relation":{},"ISSN":["1568-9972"],"issn-type":[{"value":"1568-9972","type":"print"}],"subject":[],"published":{"date-parts":[[2025,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Managing the clinical heterogeneity of patients with Still's disease, from early diagnosis to timely treatment","name":"articletitle","label":"Article Title"},{"value":"Autoimmunity Reviews","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.autrev.2025.103880","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 The Authors. Published by Elsevier B.V.","name":"copyright","label":"Copyright"}],"article-number":"103880"}}