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Autoimmun."},{"key":"10.1016\/j.smim.2022.101632_bib70","doi-asserted-by":"crossref","first-page":"124","DOI":"10.3390\/ph11040124","article-title":"Emerging and dynamic biomedical uses of ferritin","author":"Chiou","year":"2018","journal-title":"Pharmaceuticals"},{"issue":"5","key":"10.1016\/j.smim.2022.101632_bib71","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1016\/j.autrev.2015.01.004","article-title":"Increased level of H-ferritin and its imbalance with L-ferritin, in bone marrow and liver of patients with adult onset Still\u2019s disease, developing macrophage activation syndrome, correlate with the severity of the disease","volume":"14","author":"Ruscitti","year":"2015","journal-title":"Autoimmun. Rev."},{"issue":"1","key":"10.1016\/j.smim.2022.101632_bib72","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1111\/cei.12826","article-title":"Giacomelli R. 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Dis."},{"issue":"3","key":"10.1016\/j.smim.2022.101632_bib75","doi-asserted-by":"crossref","first-page":"887","DOI":"10.1002\/hep.22716","article-title":"Ferritin functions as a proinflammatory cytokine via iron-independent protein kinase C zeta\/nuclear factor kappaB-regulated signaling in rat hepatic stellate cells","volume":"49","author":"Ruddell","year":"2009","journal-title":"Hepatology"},{"issue":"3","key":"10.1016\/j.smim.2022.101632_bib76","doi-asserted-by":"crossref","first-page":"887","DOI":"10.1002\/hep.22716","article-title":"Ferritin functions as a proinflammatory cytokine via iron-independent protein kinase C zeta\/nuclear factor kappaB-regulated signaling in rat hepatic stellate cells","volume":"49","author":"Ruddell","year":"2009","journal-title":"Hepatology"},{"issue":"7","key":"10.1016\/j.smim.2022.101632_bib77","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1084\/jem.20042433","article-title":"TIM-2 is expressed on B cells and in liver and kidney and is a receptor for H-ferritin endocytosis","volume":"202","author":"Chen","year":"2005","journal-title":"J. Exp. Med."},{"issue":"8","key":"10.1016\/j.smim.2022.101632_bib78","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0023800","article-title":"Iron uptake mediated by binding of H-ferritin to the TIM-2 receptor in mouse cells","volume":"6","author":"Han","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.smim.2022.101632_bib79","doi-asserted-by":"crossref","first-page":"131","DOI":"10.3389\/fimmu.2019.00131","article-title":"Ferritin light chain confers protection against sepsis-induced inflammation and organ injury","volume":"10","author":"Zarjou","year":"2019","journal-title":"Front Immunol."},{"issue":"1","key":"10.1016\/j.smim.2022.101632_bib80","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. 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Rheumatol."},{"issue":"8","key":"10.1016\/j.smim.2022.101632_bib83","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":"5","key":"10.1016\/j.smim.2022.101632_bib84","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1002\/acr.21901","article-title":"CRI (Club Rhumatismes et Inflammation). 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Rheumatol."},{"key":"10.1016\/j.smim.2022.101632_bib91","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.bbi.2018.02.013","article-title":"Rethinking IL-6 and CRP: why they are more than inflammatory biomarkers, and why it matters","volume":"70","author":"Del Giudice","year":"2018","journal-title":"Brain Behav. Immun."},{"issue":"4","key":"10.1016\/j.smim.2022.101632_bib92","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1158\/2326-6066.CIR-14-0022","article-title":"The biology and medical implications of interleukin-6","volume":"2","author":"Tanaka","year":"2014","journal-title":"Cancer Immunol. Res."},{"key":"10.1016\/j.smim.2022.101632_bib93","doi-asserted-by":"crossref","first-page":"2607","DOI":"10.3389\/fimmu.2018.02607","article-title":"Pentraxins and Fc receptor-mediated immune responses","volume":"9","author":"Lu","year":"2018","journal-title":"Front Immunol."},{"issue":"6","key":"10.1016\/j.smim.2022.101632_bib94","doi-asserted-by":"crossref","first-page":"1403","DOI":"10.1016\/j.jhep.2016.02.004","article-title":"IL-6 pathway in the liver: from physiopathology to therapy","volume":"64","author":"Schmidt-Arras","year":"2016","journal-title":"J. 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Biol. Ther."},{"issue":"12","key":"10.1016\/j.smim.2022.101632_bib103","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":"1","key":"10.1016\/j.smim.2022.101632_bib104","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1002\/acr.20319","article-title":"Club Rhumatismes Et Inflammation. 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Rheumatol."},{"issue":"2","key":"10.1016\/j.smim.2022.101632_bib109","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1097\/00005792-199103000-00004","article-title":"Adult Still's disease: manifestations, disease course, and outcome in 62 patients","volume":"70","author":"Pouchot","year":"1991","journal-title":"Medicine"},{"issue":"1","key":"10.1016\/j.smim.2022.101632_bib110","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1186\/s12916-016-0738-8","article-title":"Adult-onset Still's 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":"1","key":"10.1016\/j.smim.2022.101632_bib111","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1186\/s13075-020-02245-5","article-title":"Parenchymal lung disease in adult onset Still's 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":"6","key":"10.1016\/j.smim.2022.101632_bib112","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. 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