Presentation and diagnosis of adult-onset Still’s disease: the implications of current and emerging markers in overcoming the diagnostic challenge

References

• Bywaters EG. Still’s disease in the adult. Ann Rheum Dis 1971;30:121-33
   PubMed Web of Science ®Google Scholar
• Still GF. On a form of chronic joint disease in children. Med Chir Trans 1897;80:47-60
   PubMedGoogle Scholar
• Magadur-Joly G, Billaud E, Barrier JH, et al. Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 1995;54:587-90
   PubMed Web of Science ®Google Scholar
• Wakai K, Ohta A, Tamakoshi A, et al. Estimated prevalence and incidence of adult Still’s disease: findings by a nationwide epidemiological survey in Japan. J Epidemiol 1997;7:221-5
   PubMedGoogle Scholar
• Evensen KJ, Nossent HC. Epidemiology and outcome of adult-onset Still’s disease in Northern Norway. Scand J Rheumatol 2006;35:48-51
   PubMed Web of Science ®Google Scholar
• Efthimiou P, Moorthy LN, Mavragani CP, et al. Adult Onset Still’s Disease and Autoinflammation. Int J Inflam 2012;2012:964751
   PubMedGoogle Scholar
• Gerfaud-Valentin M, Jamilloux Y, Iwaz J, et al. Adult-onset Still’s disease. Autoimmun Rev 2014;13(7):708-22
   PubMed Web of Science ®Google Scholar
• Maria AT, Le Quellec A, Jorgensen C, et al. Adult onset Still’s disease (AOSD) in the era of biologic therapies: Dichotomous view for cytokine and clinical expressions. Autoimmun Rev 2014;13(11):1149-59
   PubMed Web of Science ®Google Scholar
• Rosário C, Zandman-Goddard G, Meyron-Holtz EG, et al. The hyperferritinemic syndrome: macrophage activation syndrome, Still’s disease, septic shock and catastrophic antiphospholipid syndrome. BMC Med 2013;11:185
   PubMed Web of Science ®Google Scholar
• Pouchot J, Sampalis JS, Beaudet F, et al. Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine Baltimore 1991;70:118-36
   PubMed Web of Science ®Google Scholar
• Fujii T, Nojima T, Yasuoka H, et al. Cytokine and immunogenetic profiles in Japanese patients with adult Still’s disease. Association with chronic articular disease. Rheumatology (Oxford) 2001;40:1398-404
   PubMed Web of Science ®Google Scholar
• Joung CI, Lee HS, Lee SW, et al. Association between HLADR B1 and clinical features of adult onset Still’s disease in Korea. Clin Exp Rheumatol 2003;21:489-92
   PubMed Web of Science ®Google Scholar
• Wang FF, Huang XF, Shen N, et al. A genetic role for macrophage migration inhibitory factor (MIF) in adult-onset Still’s disease. Arthritis Res Ther 2013;15(3):R65
   PubMed Web of Science ®Google Scholar
• Nonaka F, Migita K, Jiuchi Y, et al. Increased prevalence of MEFV exon 10 variants in Japanese patients with adult onset Still’s disease. Clin Exp Immunol 2015;179(3):392-7
   PubMedGoogle Scholar
• Sugiura T, Maeno N, Kawaguchi Y, et al. A promoter haplotype of the interleukin-18 gene is associated with juvenile idiopathic arthritis in the Japanese population. Arthritis Res Ther 2006;8:R60
   PubMed Web of Science ®Google Scholar
• Balleari E, Cutolo M, Accardo S. Adult-onset Still’s disease associated to toxoplasma gondii infection. Clin Rheumatol 1991;10(3):326-7
   PubMedGoogle Scholar
• Yilmaz S, Karakas A, Cinar M, et al. Adult onset Still’s disease as a paraneoplastic syndrome–a case report and review of the literature. Bull Hosp Jt Dis 2013;71(2):156-60
   Google Scholar
• Chen DY, Lan JL, Lin FJ, et al. Predominance of Th1 cytokine in peripheral blood and pathological tissues of patients with active untreated adult onset Still’s disease. Ann Rheum Dis 2004;63(10):1300-6
   PubMedGoogle Scholar
• Chen DY, Lan JL, Lin FJ, et al. Proinflammatory cytokine profiles in sera and pathological tissues of patients with active untreated adult onset Still’s disease. J Rheumatol 2004;31:2189-98
   PubMed Web of Science ®Google Scholar
• Colafrancesco S, Priori R, Alessandri C, et al. IL-18 Serum level in adult onset still’s disease: a marker of disease activity. Int J Inflam 2012;2012:156890
   PubMedGoogle Scholar
• Matsui K, Tsuchida T, Hiroishi K, et al. High serum level of macrophage-colony stimulating factor (M-CSF) in adult-onset Still’s disease. Rheumatology (Oxford) 1999;38(5):477-8
   PubMedGoogle Scholar
• Crispin JC, Martinez-Banos D, Alcocer-Varela J. Adult-onset still disease as the cause of fever of unknown origin. Medicine (Baltimore) 2005;84:331-7
   PubMed Web of Science ®Google Scholar
• Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis 2006;65(5):564-72
   PubMed Web of Science ®Google Scholar
• Priori R, Colafrancesco S, Gattamelata A, et al. Adult onset Still Disease: a rare disorder with potentially fatal outcome. Autoimmun Highlights 2010;1:53-9
   PubMedGoogle Scholar
• Cozzi A, Papagrigoraki A, Biasi D, et al. Cutaneous manifestations of adult-onset Still’s disease: a case report and review of literature. Clin Rheumatol 2014. [Epub ahead of print]
   PubMedGoogle Scholar
• Yamamoto T. Cutaneous manifestations associated with adult onset Still’s disease: important diagnostic values. Rheumatol Int 2012;32(8):2233-7
   PubMed Web of Science ®Google Scholar
• Kontzias A, Efthimiou P. Adult-onset Still’s disease: pathogenesis, clinical manifestations and therapeutic advances. Drugs 2008;68(3):319-37
   PubMed Web of Science ®Google Scholar
• Priori R, Barone F, Alessandri C, et al. Markedly increased IL-18 liver expression in adult-onset Still’s disease-related hepatitis. Rheumatology (Oxford) 2011;50(4):776-80
   PubMed Web of Science ®Google Scholar
• Dino O, Provenzano G, Giannuoli G, et al. Fulminant hepatic failure in adult onset Still’s disease. J Rheumatol 1996;23:784-5
   PubMed Web of Science ®Google Scholar
• Fautrel B. Adult-onset Still disease. Best Pract Res Clin Rheumatol 2008;22(5):773-92
   PubMed Web of Science ®Google Scholar
• Ichida H, Kawaguchi Y, Sugiura T, et al. Clinical manifestations of adult-onset still’s disease presenting with erosive arthritis: Association with low levels of ferritin and IL-18. Arthritis Care Res Hoboken 2013; Epub ahead of print
   Google Scholar
• Colina M, Trotta F. Clinical predictors in adult-onset Still disease: there is no getting away from ferritin and arthritis. J Dermatol 2013;40(3):234-5
   PubMedGoogle Scholar
• Efthimiou P, Kadavath S, Mehta B. Life-threatening complications of adult-onset Still’s disease. Clin Rheumatol 2014;33(3):305-14
   PubMed Web of Science ®Google Scholar
• Priori R, Colafrancesco S, Picarelli G, et al. Adult-onset Still’s disease: not always so good. Clin Exp Rheumatol 2012;30(1):142
   PubMed Web of Science ®Google Scholar
• Rau M, Schiller M, Krienke S, et al. Clinical manifestations but not cytokine profiles differentiate adult-onset-still’s disease and sepsis. J Rheumatol 2010;37(11):2369-76
   PubMed Web of Science ®Google Scholar
• Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992;19(3):424-30
   PubMed Web of Science ®Google Scholar
• Cush JJ, Medsger TAJr, Christy WC, et al. Adult-onset Still’s disease: clinical course and outcome. Arthritis Rheum 1987;30(2):186-94
   PubMedGoogle Scholar
• Fautrel B, Zing E, Golmard JL, et al. Proposal for a new set of classification criteria for adult-onset Still disease. Medicine (Baltimore) 2002;81(3):194-200
   PubMed Web of Science ®Google Scholar
• Dellinger RP, Levy MM, Rhodes A, et al. Surviving Sepsis Campaign: international guidelines for management of severe sepsis and septic shock, 2012. Intensive Care Med 2013;39(2):165-228
   PubMed Web of Science ®Google Scholar
• Prucha M, Bellingan G, Zazula R. Sepsis biomarkers. Clin Chim Acta 2014;440C:97-103
   Google Scholar
• Wu N, Li Q, Gu CX, et al. Paraneoplastic syndrome mimicking adult onset Still’s disease caused by advanced lung cancer: a case report. BMC Cancer 2011;11:487
   PubMed Web of Science ®Google Scholar
• Inoue R, Kato T, Kim F, et al. A case of adult onset Still’s disease (AOSD)-like manifestations abruptly developing during confirmation of a diagnosis of metastatic papillary thyroid carcinoma. Mod Rheumatol 2012;22:796-800
   PubMedGoogle Scholar
• Voinchet H, Etienne G, Ghiringelli CB, et al. Lymphomes spléniques de la zone marginale et auto-immunité : à propos de 6 cas. Rev Med Interne 2010;31:4-11
   PubMed Web of Science ®Google Scholar
• Mekinian A, Lambert M, Queyrel V, et al. Maladie de Still de l’adulte et angiosarcome hépatique, une association fortuite ou un syndrome paranéoplasique : à propos d’un cas. Rev Med Interne 2008;29:936-9
   PubMedGoogle Scholar
• Shibuya Y, Matuo K, Kawada T, et al. Adult onset Still’s disease associated esophageal cancer: a case report. Ryumachi 2003;43:577-82
   PubMedGoogle Scholar
• Neishi J, Tsukada Y, Maehara T, et al. Adult Still’s disease as a paraneoplastic manifestation of breast cancer. Scand J Rheumatol 2000;29:328-30
   PubMed Web of Science ®Google Scholar
• Liozon E, Ly KH, Vidal-Cathala E, et al. Adult-onset Still’s disease as a manifestation of malignancy: report of a patient with melanoma and literature review. Rev Med Interne 2014;35(1):60-4
   PubMed Web of Science ®Google Scholar
• Yilmaz S, Karakas A, Cinar M, et al. Adult onset Still’s disease as a paraneoplastic syndrome–a case report and review of the literature. Bull Hosp Jt Dis 2013;71(2):156-60
   Google Scholar
• Ota T, Higashi S, Suzuki H, Eto S. Increased serum ferritin levels in adult Still’s disease. Lancet 1987;1(8532):562-3
   PubMed Web of Science ®Google Scholar
• Ohta A, Yamaguchi M, Tsunematsu T, et al. Adult Still’s disease: a multicenter survey of Japanese patients. J Rheumatol 1990;17(8):1058-63
   PubMed Web of Science ®Google Scholar
• Fautrel B, Le Moel G, Saint-Marcoux B, et al. Diagnostic value of ferritin and glycosylated ferritin in adult onset still’s disease. J Rheumatol 2001;28:322-9
   PubMed Web of Science ®Google Scholar
• Lian F, Wang Y, Yang X, et al. Clinical features and hyperferritinemia diagnostic cutoff points for AOSD based on ROC curve: a Chinese experience. Rheumatol Int 2012;32:189-92
   PubMed Web of Science ®Google Scholar
• Van Reeth C, Le Moël G, Lasne Y, et al. Serum ferritin and isoferritins are tools for diagnosis of active Still’s disease. J Rheumatol 1994;21:890-5
   PubMed Web of Science ®Google Scholar
• Vignes S, Le Moël G, Fautrel B, et al. Percentage of glycosylated serum ferritin remains low throughout the course of adult onset Still’s disease. Ann Rheum Dis 2000;59(5):347-50
   PubMedGoogle Scholar
• Fautrel B. Ferritin levels in adult Still’s disease: any sugar? Joint Bone Spine 2002;69:355-7
   PubMedGoogle Scholar
• Zandman-Goddard G, Shoenfeld Y. Ferritin in autoimmune diseases. Autoimmun Rev 2007;6:457-63
   PubMed Web of Science ®Google Scholar
• Mehta B, Efthimiou P. Ferritin in adult-onset still’s disease: just a useful innocent bystander? Int J Inflam 2012;2012:298405
   PubMedGoogle Scholar
• Recalcati S, Invernizzi P, Arosio P, et al. New functions for an iron storage protein: the role of ferritin in immunity and autoimmunity. J Autoimmun 2008;30:84-9
   PubMed Web of Science ®Google Scholar
• Chen TT, Li L, Chung DH, et al. TIM-2 is expressed on B cells and in liver and kidney and is a receptor for H-ferritin endocytosis. J Exp Med 2005;202:955-65
   PubMed Web of Science ®Google Scholar
• Colafrancesco S, Priori R, Alessandri C, et al. sCD163 in AOSD: a biomarker for macrophage activation related to hyperferritinemia. Immunol Res 2014;60(2-3):177-83
   PubMed Web of Science ®Google Scholar
• Cohen LA, Gutierrez L, Weiss A, et al. Serum ferritin is derived primarily from macrophages through a nonclassical secretory pathway. Blood 2010;116:1574-84
   PubMed Web of Science ®Google Scholar
• Bombardieri M, Mc Innes IB, Pitzalis C. Interleukin 18 as potential therapeutic target in chronic autoimmune/autoinflammatory conditions. Expert Opin Biol Ther 2007;7(1):31-40
   PubMed Web of Science ®Google Scholar
• Kawashima M, Yamamura M, Taniai M, et al. Levels of interleukin-18 and its binding inhibitors in the blood circulation of patients with adult-onset Still’s disease. Arthritis Rheum 2001;44(3):550-60
   PubMedGoogle Scholar
• Kawaguchy Y, Terajima H, Harigai M, et al. Interleukin-18 as a novel diagnostic marker and indicator of disease severity in adult-onset Still’s disease. Arthritis Rheum 2001;44(7):1716-17
   PubMedGoogle Scholar
• Conigliaro P, Priori R, Bombardieri M, et al. Lymph node IL-18 expression in adult-onset Still’s disease. Ann Rheum Dis 2009;68(3):442-3
   PubMedGoogle Scholar
• Tschoeke SK, Oberholzer A, Moldawer LL. Interleukin-18: a novel prognostic cytokine in bacteria-induced sepsis. Crit Care Med 2006;34(4):1225-33
   PubMedGoogle Scholar
• Priori R, Colafrancesco S, Alessandri C, et al. Interleukin 18: a biomarker for differential diagnosis between adult-onset Still’s disease and sepsis. J Rheumatol 2014;41(6):1118-23
   PubMedGoogle Scholar
• Whicher J, Bienvenu J, Monneret G. Procalcitonin as an acute phase marker. Ann Clin Biochem 2001;38(Pt 5):483-93
   PubMedGoogle Scholar
• Meisner M. Pathobiochemistry and clinical use of procalcitonin. Clin Chim Acta 2002;323:17-29
   PubMed Web of Science ®Google Scholar
• Scirè CA, Cavagna L, Perotti C, et al. Diagnostic value of procalcitonin measurement in febrile patients with systemic autoimmune diseases. Clin Exp Rheumatol 2006;24(2):123-8
   PubMed Web of Science ®Google Scholar
• Dele`vaux I, Andre M, Colombier M, et al. Can procalcitonin measurement help in differentiating between bacterial infection and other kinds of inflammatory processes? Ann Rheum Dis 2003;62:337-40
   PubMedGoogle Scholar
• Ames PR, Walker E, Aw D, et al. Multi-organ failure in adult onset Still’s disease: a septic disguise. Clin Rheumatol 2009;28(Suppl 1):S3-6
   Google Scholar
• Chen DY, Chen YM, Ho WL, et al. Diagnostic value of procalcitonin for differentiation between bacterial infection and non-infectious inflammation in febrile patients with active adult-onset Still’s disease. Ann Rheum Dis 2009;68(6):1074-5
   PubMedGoogle Scholar
• Møller HJ. Soluble CD163. Scand J Clin Lab Invest 2012;72:1-13
   PubMed Web of Science ®Google Scholar
• Bleesing J, Prada A, Siegel DM, et al. The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor alpha-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis. Arthritis Rheum 2007;56:965-71
   PubMed Web of Science ®Google Scholar
• Schaer DJ, Schleiffenbaum B, Kurrer M, et al. Soluble hemoglobin-haptoglobin scavenger receptor CD163 as a lineage-specific marker in the reactive hemophagocytic syndrome. Eur J Haematol 2005;74:6-10
   PubMed Web of Science ®Google Scholar
• Feng L, Zhou X, Su LX, et al. Clinical significance of soluble hemoglobin scavenger receptor CD163 (sCD163) in sepsis, a prospective study. PLoS One 2012;7:e38400
   Web of Science ®Google Scholar
• Hiraoka A, Horiike N, Akbar SM, et al. Soluble CD163 in patients with liver diseases: very high levels of soluble CD163 in patients with fulminant hepatic failure. J Gastroenterol 2005;40:52-6
   PubMed Web of Science ®Google Scholar
• Cairo G, Recalcati S, Mantovani A, et al. Iron trafficking and metabolism in macrophages: contribution to the polarized phenotype. Trends Immunol 2011;32:241-7
   PubMed Web of Science ®Google Scholar
• Kessel C, Holzinger D, Foell D. Phagocyte-derived S100 proteins in autoinflammation: putative role in pathogenesis and usefulness as biomarkers. Clin Immunol 2013;147(3):229-41
   PubMed Web of Science ®Google Scholar
• Ryckman C, Vandal K, Rouleau P, et al. Proinflammatory activities of S100: proteins S100A8, S100A9, and S100A8/A9 induce neutrophil chemotaxis and adhesion. J Immunol 2003;170(6):3233-42
   PubMed Web of Science ®Google Scholar
• Yan WX, Armishaw C, Goyette J, et al. Mast cell and monocyte recruitment by S100A12 and its hinge domain. J Biol Chem 2008;283(19):13035-43
   PubMedGoogle Scholar
• Viemann D, Strey A, Janning A, et al. Myeloid-related proteins 8 and 14 induce a specific inflammatory response in human microvascular endothelial cells. Blood 2005;105(7):2955-62
   PubMed Web of Science ®Google Scholar
• Jung SY, Park YB, Ha YJ, et al. Serum calprotectin as a marker for disease activity and severity in adult-onset Still’s disease. J Rheumatol 2010;37(5):1029-34
   PubMed Web of Science ®Google Scholar
• Kim HA, An JM, Nam JY, et al. Serum S100A8/A9, but not follistatin-like protein 1 and interleukin 18, may be a useful biomarker of disease activity in adult-onset Still’s disease. J Rheumatol 2012;39(7):1399-406
   PubMedGoogle Scholar
• Bae CB, Suh CH, An JM, et al. Serum S100A12 may be a useful biomarker of disease activity in adult-onset still’s disease. J Rheumatol 2014;41(12):2403-8
   PubMedGoogle Scholar
• Martini A. Systemic juvenile idiopathic arthritis. Autoimmun Rev 2012;12(1):56-9
   PubMed Web of Science ®Google Scholar
• Martini A. It is time to rethink juvenile idiopathic arthritis classification and nomenclature. Ann Rheum Dis 2012;71(9):1437-9
   PubMed Web of Science ®Google Scholar
• Luthi F, Zufferey P, Hofer MF, et al. Adolescent-onset Still’s disease: characteristics and outcome in comparison with adult-onset Still’s disease. Clin Exp Rheumatol 2002;20(3):427-30
   PubMedGoogle Scholar
• Jamilloux Y, Gerfaud-Valentin M, Martinon F, et al. Pathogenesis of adult-onset Still’s disease: new insights from the juvenile counterpart. Immunol Res 2015;61(1-2):53-62
   PubMed Web of Science ®Google Scholar
• Mellins ED, Macaubas C, Grom AA. Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions. Nat Rev Rheumatol 2011;7(7):416-26
   PubMed Web of Science ®Google Scholar
• Covone AE, Solari N, Malattia C, et al. Periostin gene variants are associated with disease course and severity in juvenile idiopathic arthritis. Clin Exp Rheumatol 2014;32(5):747-53
   PubMedGoogle Scholar
• Grom A. Natural killer cell dysfunction: A common pathway in systemic-onset juvenile rheumatoid arthritis, macrophage activation syndrome, and hemophagocytic lymphohistiocytosis? Arthritis Rheum 2004;50:689-98
   PubMed Web of Science ®Google Scholar
• Behrens EM, Beukelman T, Paessler M, et al. Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis. J Rheumatol 2007;34:1133-8
   PubMed Web of Science ®Google Scholar
• Zhang K, Biroschak J, Glass DN, et al. Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis is associated with MUNC13–4 polymorphisms. Arthritis Rheum 2008;58:2892-6
   PubMed Web of Science ®Google Scholar
• Hazen MM, Woodward AL, Hofmann I, et al. Mutations of the hemophagocytic lymphohistiocytosis-associated gene UNC13D in a patient with systemic juvenile idiopathic arthritis. Arthritis Rheum 2008;58(2):567-70
   PubMed Web of Science ®Google Scholar
• Yanagimachi M, Naruto T, Miyamae T, et al. Association of IRF5 polymorphisms with susceptibility to macrophage activation syndrome in patients with juvenile idiopathic arthritis. J Rheumatol 2011;38:769-74
   PubMedGoogle Scholar
• Vastert SJ, van Wijk R, D’Urbano LE, et al. Mutations in the perforin gene can be linked to macrophage activation syndrome in patients with systemic onset juvenile idiopathic arthritis. Rheumatology (Oxford) 2010;49:441-9
   PubMed Web of Science ®Google Scholar
• Kimura Y, Weiss JE, Haroldson KL, et al. Pulmonary hypertension and other potentially fatal pulmonary complications in systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken) 2013;65(5):745-52
   PubMed Web of Science ®Google Scholar
• Belot A, Bader-Meunier B, Niaudet P, et al. ANCA-associated glomerulonephritis in systemic-onset juvenile idiopathic arthritis. Am J Kidney Dis 2012;59(3):439-43
   PubMedGoogle Scholar
• Consolaro A, Varnier GC, Martini A, et al. Advances in biomarkers for paediatric rheumatic diseases. Nat Rev Rheumatol 2014; [Epub ahead of print]
   PubMedGoogle Scholar
• Shimizu M, Nakagishi Y, Yachie A. Distinct subsets of patients with systemic juvenile idiopathic arthritis based on their cytokine profiles. Cytokine 2013;61(2):345-8
   PubMed Web of Science ®Google Scholar
• Bleesing J, Prada A, Siegel DM, et al. The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor alpha-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis. Arthritis Rheum 2007;56(3):965-71
   PubMed Web of Science ®Google Scholar
• Gorelik M, Fall N, Altaye M, et al. Follistatin-like protein 1 and the ferritin/erythrocyte sedimentation rate ratio are potential biomarkers for dysregulated gene expression and macrophage activation syndrome in systemic juvenile idiopathic arthritis. J Rheumatol 2013;40(7):1191-9
   PubMed Web of Science ®Google Scholar
• Quartier P. Current treatments for juvenile idiopathic arthritis. Joint Bone Spine 2010;77(6):511-16
   PubMedGoogle Scholar
• Verbsky JW, White AJ. Effective use of the recombinant interleukin 1 receptor antagonist anakinra in therapy resistant systemic onset juvenile rheumatoid arthritis. J Rheumatol 2004;31:2071-5
   PubMed Web of Science ®Google Scholar
• Pascual V, Allantaz F, Arce E, et al. Role of interleukin-1 (IL-1) in the pathogenesis of systemic onset juvenile idiopathic arthritis and clinical response to IL-1 blockade. J Exp Med 2005;201:1479-8
   PubMed Web of Science ®Google Scholar
• Fitzgerald AA, LeClercq SA, Yan A, et al. Rapid responses to anakinra in patients with refractory adult onset Still’s disease. Arthritis Rheum 2005;52:1794-803
   PubMed Web of Science ®Google Scholar
• Quartier P, Allantaz F, Cimaz R, et al. A multicentre, randomised, double-blind, placebo-controlled trial with the interleukin-1 receptor antagonist anakinra in patients with systemic-onset juvenile idiopathic arthritis (ANAJIS trial). Ann Rheum Dis 2011;70(5):747-54
   PubMed Web of Science ®Google Scholar
• Lequerré T, Quartier P, Rosellini D, et al. Interleukin-1 receptor antagonist (anakinra) treatment in patients with systemic-onset juvenile idiopathic arthritis or adult onset Still disease: preliminary experience in France. Ann Rheum Dis 2008;67(3):302-8
   PubMed Web of Science ®Google Scholar
• Vastert SJ, de Jager W, Noordman BJ, et al. 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. Arthritis Rheumatol 2014;66(4):1034-43
   PubMed Web of Science ®Google Scholar
• Ruperto N, Brunner HI, Quartier P, et al. Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis. N Engl J Med 2012;367(25):2396-406
   PubMed Web of Science ®Google Scholar
• Kontzias A, Efthimiou P. The use of Canakinumab, a novel IL-1β long-acting inhibitor, in refractory adult-onset Still’s disease. Semin Arthritis Rheum 2012;42(2):201-5
   PubMed Web of Science ®Google Scholar
• Ilowite NT, Prather K, Lokhnygina Y, et al. Randomized, double-blind, placebo-controlled trial of the efficacy and safety of rilonacept in the treatment of systemic juvenile idiopathic arthritis. Arthritis Rheumatol 2014;66(9):2570-9
   PubMed Web of Science ®Google Scholar
• Lovell DJ, Giannini EH, Reiff AO, et al. Long-term safety and efficacy of rilonacept in patients with systemic juvenile idiopathic arthritis. Arthritis Rheum 2013;65(9):2486-96
   PubMed Web of Science ®Google Scholar
• Gattorno M, Piccini A, Lasigliè D, et al. The pattern of response to anti-interleukin-1 treatment distinguishes two subsets of patients with systemic-onset juvenile idiopathic arthritis. Arthritis Rheum 2008;58(5):1505-15
   PubMed Web of Science ®Google Scholar
• Nordstrom D, Knight A, Luukkainen R, et al. Beneficial effect of interleukin 1 inhibition with anakinra in adult onset Still’s disease. An open, randomized, multi center study. J Rheumatol 2012;39:200
   Google Scholar
• Lo Gullo A, Caruso A, Pipitone N, et al. Canakinumab in a case of adult onset still’s disease: efficacy only on systemic manifestations. Joint Bone Spine 2014;81(4):376-7
   PubMed Web of Science ®Google Scholar
• Barsotti S, Neri R, Iacopetti V, et al. Successful treatment of refractory adult-onset still disease with canakinumab: a case report. J Clin Rheumatol 2014;20(2):121
   PubMed Web of Science ®Google Scholar
• Cagatay Y, Gul A, Cagatay A, et al. Adult-onset Still’s disease. Int J Clin Pract 2009;63(7):1050-5
   PubMedGoogle Scholar
• Colina M, Zucchini W, Ciancio G, et al. The evolution of adult-onset Still disease: an observational and comparative study in a cohort of 76 Italian patients. Semin Arthritis Rheum 2011;41(2):279-85
   PubMed Web of Science ®Google Scholar
• Mehrpoor G, Owlia MB, Soleimani H, Ayatollahi J. Adult-onset Still’s disease: a report of 28 cases and review of the literature. Mod Rheumatol 2008;18(5):480-5
   PubMedGoogle Scholar
• Riera E, Olivé A, Narváez J, et al. Adult onset Still’s disease: review of 41 cases. Clin Exp Rheumatol 2011;29(2):331-6
   PubMedGoogle Scholar