Adult-onset Still disease

References

Key articles

Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still's disease. J Autoimmun. 2018 Sep;93:24-36.Full text  Abstract

Vordenbäumen S, Feist E, Rech J, et al. Diagnosis and treatment of adult-onset Still's disease: a concise summary of the German society of rheumatology S2 guideline. Z Rheumatol. 2023 Feb;82(suppl 2):81-92.Full text  Abstract

Macovei LA, Burlui A, Bratoiu I, et al. Adult-onset Still's disease-a complex disease, a challenging treatment. Int J Mol Sci. 2022 Oct 24;23(21):12810.Full text  Abstract

Efthimiou P, Kontzias A, Hur P, et al. Adult-onset Still's disease in focus: clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Semin Arthritis Rheum. 2021 Aug;51(4):858-74.Full text  Abstract

Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992 Mar;19(3):424-30. Abstract

Reference articles

1. Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still's disease. J Autoimmun. 2018 Sep;93:24-36.Full text  Abstract

2. Vordenbäumen S, Feist E, Rech J, et al. Diagnosis and treatment of adult-onset Still's disease: a concise summary of the German society of rheumatology S2 guideline. Z Rheumatol. 2023 Feb;82(suppl 2):81-92.Full text  Abstract

3. Colafrancesco S, Manara M, Bortoluzzi A, et al. Management of adult-onset Still's disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts. Arthritis Res Ther. 2019 Dec 11;21(1):275.Full text  Abstract

4. Macovei LA, Burlui A, Bratoiu I, et al. Adult-onset Still's disease-a complex disease, a challenging treatment. Int J Mol Sci. 2022 Oct 24;23(21):12810.Full text  Abstract

5. Efthimiou P, Kontzias A, Hur P, et al. Adult-onset Still's disease in focus: clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Semin Arthritis Rheum. 2021 Aug;51(4):858-74.Full text  Abstract

6. Al-Hakim A, Mistry A, Savic S. Improving diagnosis and clinical management of acquired systemic autoinflammatory diseases. J Inflamm Res. 2022;15:5739-55.Full text  Abstract

7. Sfriso P, Priori R, Valesini G, et al. Adult-onset Still's disease: an Italian multicentre retrospective observational study of manifestations and treatments in 245 patients. Clin Rheumatol. 2016 Jul;35(7):1683-9.Full text  Abstract

8. Bogdan M, Nitsch-Osuch A, Samel-Kowalik P, et al. Adult-onset Still's disease in Poland - a nationwide population-based study. Ann Agric Environ Med. 2021 Jun 14;28(2):250-4.Full text  Abstract

9. 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 Jul;54(7):587-90.Full text  Abstract

10. Evensen KJ, Nossent HC. Epidemiology and outcome of adult-onset Still's disease in Northern Norway. Scand J Rheumatol. 2006 Jan-Feb;35(1):48-51.Full text  Abstract

11. Lenert A, Oh G, Ombrello MJ, et al. Clinical characteristics and comorbidities in adult-onset Still's disease using a large US administrative claims database. Rheumatology (Oxford). 2020 Jul 1;59(7):1725-33.Full text  Abstract

12. Mehta BY, Ibrahim S, Briggs W, et al. Racial/ethnic variations in morbidity and mortality in adult onset Still's disease: an analysis of national dataset. Semin Arthritis Rheum. 2019 Dec;49(3):469-73.Full text  Abstract

13. Magliulo D, Narayan S, Ue F, et al. Adult-onset Still's disease after mRNA COVID-19 vaccine. Lancet Rheumatol. 2021 Oct;3(10):e680-2.Full text  Abstract

14. Palassin P, Bres V, Hassan S, et al. Comprehensive description of adult-onset Still's disease after COVID-19 vaccination. J Autoimmun. 2023 Jan;134:102980.Full text  Abstract

15. Matsuda M, Funakubo Asanuma Y, Yokota K, et al. New-onset adult-onset Still's disease following COVID-19 vaccination: three case reports and a literature review. Intern Med. 2023 Jan 15;62(2):299-305.Full text  Abstract

16. Balci MA, Pamuk ON, Pamuk GE, et al. AB1142 epidemiology and outcome of adult-onset Still’s disease in Northwestern Thrace region in Turkey. Ann Rheum Dis. 2015;74(suppl 2):1284.

17. Hocevar A, Rotar Z, Krosel M, et al. SAT0524 the incidence rate of adult onset Still's disease in Slovenia.Ann Rheum Dis. 2020;79 (suppl 1):1218-9.Full text

18. Wouters JM, van der Veen J, van de Putte LB, et al. Adult onset Still's disease and viral infections. Ann Rheum Dis. 1988 Sep;47(9):764-7.Full text  Abstract

19. Escudero FJ, Len O, Falcó V, et al. Rubella infection in adult onset Still's disease. Ann Rheum Dis. 2000 Jun;59(6):493.Full text  Abstract

20. Jia J, Shi H, Liu M, et al. Cytomegalovirus infection may trigger adult-onset Still's disease onset or relapses. Front Immunol. 2019;10:898.Full text  Abstract

21. Perez C, Artola V. Adult Still's disease associated with Mycoplasma pneumoniae infection. Clin Infect Dis. 2001 Mar 15;32(6):E105-6.Full text  Abstract

22. National Health Service. Clinical Commissioning Policy: tocilizumab for the treatment of adult-onset Still’s disease refractory to second-line therapy (adults) [210801P] (URN: 1609). 2021 [internet publication].Full text

23. Franchini S, Dagna L, Salvo F, et al. Adult onset Still's disease: clinical presentation in a large cohort of Italian patients. Clin Exp Rheumatol. 2010 Jan-Feb;28(1):41-8.Full text  Abstract

24. Gerfaud-Valentin M, Jamilloux Y, Iwaz J, et al. Adult-onset Still's disease. Autoimmun Rev. 2014 Jul;13(7):708-22.Full text  Abstract

25. Hu QY, Zeng T, Sun CY, et al. Clinical features and current treatments of adult-onset Still's disease: a multicentre survey of 517 patients in China. Clin Exp Rheumatol. 2019 Nov-Dec;37(6 suppl 121):52-7.Full text  Abstract

26. Kim YJ, Koo BS, Kim YG, et al. Clinical features and prognosis in 82 patients with adult-onset Still's disease. Clin Exp Rheumatol. 2014 Jan-Feb;32(1):28-33. Abstract

27. Pay S, Türkçapar N, Kalyoncu M, et al. A multicenter study of patients with adult-onset Still's disease compared with systemic juvenile idiopathic arthritis. Clin Rheumatol. 2006 Sep;25(5):639-44.Full text  Abstract

28. Zhang Y, Yang Y, Bai Y, et al. Clinical characteristics and follow-up analysis of adult-onset Still's disease complicated by hemophagocytic lymphohistiocytosis. Clin Rheumatol. 2016 May;35(5):1145-51.Full text  Abstract

29. Asanuma YF, Mimura T, Tsuboi H, et al. Nationwide epidemiological survey of 169 patients with adult Still's disease in Japan. Mod Rheumatol. 2015 May;25(3):393-400.Full text  Abstract

30. Kalyoncu U, Solmaz D, Emmungil H, et al. 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 May;69:59-63.Full text  Abstract

31. Nakamura H, Fujieda Y, Tarumi M, et al. Calcineurin inhibitors for adult-onset Still's disease: a multicentre retrospective cohort study. Clin Exp Rheumatol. 2020 Sep-Oct;38(5 suppl 127):11-6. Abstract

32. Liu Z, Lv X, Tang G. Clinical features and prognosis of adult-onset Still's disease: 75 cases from China. Int J Clin Exp Med. 2015;8(9):16634-9.Full text  Abstract

33. Riera E, Olivé A, Narváez J, et al. Adult onset Still's disease: review of 41 cases. Clin Exp Rheumatol. 2011 Mar-Apr;29(2):331-6. Abstract

34. Zeng T, Zou YQ, Wu MF, et al. Clinical features and prognosis of adult-onset still's disease: 61 cases from China. J Rheumatol. 2009 May;36(5):1026-31.Full text  Abstract

35. Chen DY, Lan JL, Hsieh TY, et al. Clinical manifestations, disease course, and complications of adult-onset Still's disease in Taiwan. J Formos Med Assoc. 2004 Nov;103(11):844-52. Abstract

36. Kong XD, Xu D, Zhang W, et al. Clinical features and prognosis in adult-onset Still's disease: a study of 104 cases. Clin Rheumatol. 2010 Sep;29(9):1015-9. Abstract

37. Kishida D, Ichikawa T, Takamatsu R, et al. Clinical characteristics and treatment of elderly onset adult-onset Still's disease. Sci Rep. 2022 Apr 26;12(1):6787.Full text  Abstract

38. Di Cola I, Di Muzio C, Conforti A, et al. Adult-onset Still's disease with elderly onset: results from a multicentre study. Clin Exp Rheumatol. 2022 Sep;40(8):1517-25.Full text  Abstract

39. Hofheinz K, Schett G, Manger B. Adult onset Still's disease associated with malignancy - cause or coincidence? Semin Arthritis Rheum. 2016 Apr;45(5):621-6.Full text  Abstract

40. 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 [in French]. Rev Med Interne. 2014 Jan;35(1):60-4.Full text  Abstract

41. Gaggiano C, Rigante D, Vitale A, et al. Hints for genetic and clinical differentiation of adult-onset monogenic autoinflammatory diseases. Mediators Inflamm. 2019;2019:3293145.Full text  Abstract

42. Cush JJ, Medsger TA Jr, Christy WC, et al. Adult-onset Still's disease. Clinical course and outcome. Arthritis Rheum. 1987 Feb;30(2):186-94.Full text  Abstract

43. Mitrovic S, Fautrel B. Clinical phenotypes of adult-onset Still's disease: new insights from pathophysiology and literature findings. J Clin Med. 2021 Jun 15;10(12):2633.Full text  Abstract

44. Nigrovic PA, Schneider R. Systemic juvenile idiopathic arthritis and adult onset still disease. In: Hashkes PJ, Laxer RM, Simon, A, eds. Textbook of autoinflammation. Cham, Switzerland: Springer Nature Switzerland AG; 2019, 587-616.

45. Gracia-Ramos AE, Contreras-Ortíz JA. Myocarditis in adult-onset Still's disease: case-based review. Clin Rheumatol. 2020 Mar;39(3):933-47. Abstract

46. Colafrancesco S, Priori R, Valesini G. Presentation and diagnosis of adult-onset Still's disease: the implications of current and emerging markers in overcoming the diagnostic challenge. Expert Rev Clin Immunol. 2015 Jun;11(6):749-61.Full text  Abstract

47. Mitrovic S, Fautrel B. New markers for adult-onset Still's disease. Joint Bone Spine. 2018 May;85(3):285-93.Full text  Abstract

48. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992 Mar;19(3):424-30. Abstract

49. Fautrel B, Zing E, Golmard JL, et al. Proposal for a new set of classification criteria for adult-onset still disease. Medicine (Baltimore). 2002 May;81(3):194-200.Full text  Abstract

50. Lebrun D, Mestrallet S, Dehoux M, et al. Validation of the Fautrel classification criteria for adult-onset Still's disease. Semin Arthritis Rheum. 2018 Feb;47(4):578-85.Full text  Abstract

51. Inoue N, Shimizu M, Tsunoda S, et al. Cytokine profile in adult-onset Still's disease: comparison with systemic juvenile idiopathic arthritis. Clin Immunol. 2016 Aug;169:8-13.Full text  Abstract

52. 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 Feb;61(1-2):53-62.Full text  Abstract

53. Ahmed MG. Clinical significance of interleukin-18 and interleukin-6 on disease course of adult-onset still’s disease. Ann Rheum Dis, 2018;77(suppl 2):1162.

54. Holzinger D, Frosch M, Kastrup A, et al. The Toll-like receptor 4 agonist MRP8/14 protein complex is a sensitive indicator for disease activity and predicts relapses in systemic-onset juvenile idiopathic arthritis. Ann Rheum Dis. 2012 Jun;71(6):974-80.Full text  Abstract

55. Aljaberi N, Tronconi E, Schulert G, et al. The use of S100 proteins testing in juvenile idiopathic arthritis and autoinflammatory diseases in a pediatric clinical setting: a retrospective analysis. Pediatr Rheumatol Online J. 2020 Jan 16;18(1):7.Full text  Abstract

56. 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 Jul;39(7):1399-406.Full text  Abstract

57. Kim HA, Han JH, Kim WJ, et al. TLR4 endogenous ligand S100A8/A9 levels in adult-onset Still's disease and their association with disease activity and clinical manifestations. Int J Mol Sci. 2016 Aug 16;17(8):1342.Full text  Abstract

58. Wittkowski H, et al. S100A12 is a novel molecular marker differentiating systemic-onset juvenile idiopathic arthritis from other causes of fever of unknown origin. Arthritis Rheum. 2008 Dec;58(12):3924-31.

59. 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 Dec;41(12):2403-8.Full text  Abstract

60. Ombrello MJ, Remmers EF, Tachmazidou I, et al. HLA-DRB1*11 and variants of the MHC class II locus are strong risk factors for systemic juvenile idiopathic arthritis. Proc Natl Acad Sci U S A. 2015 Dec 29;112(52):15970-5.Full text  Abstract

61. Teng JL, Chen X, Chen J, et al. The amino acid variants in HLA II molecules explain the major association with adult-onset Still's disease in the Han Chinese population. J Autoimmun. 2021 Jan;116:102562.Full text  Abstract

62. Bojan A, Parvu A, Zsoldos IA, et al. Macrophage activation syndrome: a diagnostic challenge (review). Exp Ther Med. 2021 Aug;22(2):904.Full text  Abstract

63. Lerkvaleekul B, Vilaiyuk S. Macrophage activation syndrome: early diagnosis is key. Open Access Rheumatol. 2018;10:117-28.Full text  Abstract

64. National Institute for Health and Care Excellence. Procalcitonin testing for diagnosing and monitoring sepsis (ADVIA Centaur BRAHMS PCT assay, BRAHMS PCT Sensitive Kryptor assay, Elecsys BRAHMS PCT assay, LIAISON BRAHMS PCT assay and VIDAS BRAHMS PCT assay) [DG18]. Oct 2015 [internet publication].Full text

65. Aho K, Palusuo T, Kurki P. Marker antibodies of rheumatoid arthritis: diagnostic and pathogenetic implications. Semin Arthritis Rheum. 1994 Jun;23(6):379-87. Abstract

66. Goldbach-Mansky R, Lee J, McCoy A, et al. Rheumatoid arthritis associated autoantibodies in patients with synovitis of recent onset. Arthritis Res. 2000;2(3):236-43.Full text  Abstract

67. Zhou X, Li Y, Wang Q. FDG PET/CT used in identifying adult-onset Still's disease in connective tissue diseases. Clin Rheumatol. 2020 Sep;39(9):2735-2742.Full text  Abstract

68. Holzinger D, Foell D, Kessel C. The role of S100 proteins in the pathogenesis and monitoring of autoinflammatory diseases. Mol Cell Pediatr. 2018 Sep 25;5(1):7.Full text  Abstract

69. Metcalfe JZ, Everett CK, Steingart KR, et al. Interferon-γ release assays for active pulmonary tuberculosis diagnosis in adults in low- and middle-income countries: systematic review and meta-analysis. J Infect Dis. 2011 Nov 15;204(suppl 4):S1120-9.Full text  Abstract

70. Hamm RL. Drug-hypersensitivity syndrome: diagnosis and treatment. J Am Coll Clin Wound Spec. 2011 Dec;3(4):77-81.Full text  Abstract

71. Barzilai A, Langevitz P, Goldberg I, et al. Erysipelas-like erythema of familial Mediterranean fever: clinicopathologic correlation. J Am Acad Dermatol. 2000 May;42(5 pt 1):791-5.Full text  Abstract

72. Japanese Ministry of Health, Labour and Welfare Clinical Practice Guidelines Committee. Evidence-based clinical practice guideline for adult Still’s disease​. 2018 [internet publication].Full text

73. Fautrel B, Patterson J, Bowe C, et al. Systematic review on the use of biologics in adult-onset still's disease. Semin Arthritis Rheum. 2023 Feb;58:152139.Full text  Abstract

74. Galozzi P, Bindoli S, Doria A, et al. Progress in biological therapies for adult-onset Still's disease. Biologics. 2022;16:21-34.Full text  Abstract

75. 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 Oct;41(2):279-85.Full text  Abstract

76. Fautrel B, Borget C, Rozenberg S, et al. Corticosteroid sparing effect of low dose methotrexate treatment in adult Still's disease. J Rheumatol. 1999 Feb;26(2):373-8. Abstract

77. Cipriani P, Ruscitti P, Carubbi F, et al. Methotrexate in rheumatoid arthritis: optimizing therapy among different formulations. Current and emerging paradigms. Clin Ther. 2014 Mar 1;36(3):427-35. Abstract

78. Sfriso P, Bindoli S, Galozzi P. Adult-onset Still's disease: molecular pathophysiology and therapeutic advances. Drugs. 2018 Aug;78(12):1187-95. Abstract

79. Mitamura M, Tada Y, Koarada S, et al. Cyclosporin A treatment for Japanese patients with severe adult-onset Still's disease. Mod Rheumatol. 2009;19(1):57-63.Full text  Abstract

80. Jamilloux Y, Gerfaud-Valentin M, Henry T, et al. Treatment of adult-onset Still's disease: a review. Ther Clin Risk Manag. 2015;11:33-43.Full text  Abstract

81. Kaneko Y. Interluekin-6 inhibitors for the treatment of adult-onset Still's disease. Mod Rheumatol. 2022 Jan 5;32(1):12-5.Full text  Abstract

82. Colafrancesco S, Priori R, Valesini G, et al. Response to interleukin-1 inhibitors in 140 Italian patients with adult-onset Still's disease: a multicentre retrospective observational study. Front Pharmacol. 2017;8:369.Full text  Abstract

83. Zhou S, Qiao J, Bai J, et al. Biological therapy of traditional therapy-resistant adult-onset Still's disease: an evidence-based review. Ther Clin Risk Manag. 2018;14:167-71.Full text  Abstract

84. Laskari K, Tektonidou MG, Katsiari C, et al. Outcome of refractory to conventional and/or biologic treatment adult Still's disease following canakinumab treatment: countrywide data in 50 patients. Semin Arthritis Rheum. 2021 Feb;51(1):137-43.Full text  Abstract

85. Ruscitti P, Ursini F, Sota J, et al. The reduction of concomitant glucocorticoids dosage following treatment with IL-1 receptor antagonist in adult onset Still's disease. A systematic review and meta-analysis of observational studies. Ther Adv Musculoskelet Dis. 2020;12:1759720X20933133.Full text  Abstract

86. Fautrel B, Sibilia J, Mariette X, et al. Tumour necrosis factor alpha blocking agents in refractory adult Still's disease: an observational study of 20 cases. Ann Rheum Dis. 2005 Feb;64(2):262-6.Full text  Abstract

87. Carter SJ, Tattersall RS, Ramanan AV. Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment. Rheumatology (Oxford). 2019 Jan 1;58(1):5-17.Full text  Abstract

88. Hines MR, von Bahr Greenwood T, Beutel G, et al. Consensus-based guidelines for the recognition, diagnosis, and management of hemophagocytic lymphohistiocytosis in critically ill children and adults. Crit Care Med. 2022 May 1;50(5):860-72. Abstract

89. Mehta P, Cron RQ, Hartwell J, et al. Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndrome. Lancet Rheumatol. 2020 Jun;2(6):e358-67.Full text  Abstract

90. La Rosée P, Horne A, Hines M, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019 Jun 6;133(23):2465-77.Full text  Abstract

91. Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still's disease. Nat Rev Rheumatol. 2018 Oct;14(10):603-18.Full text  Abstract

92. Pouchot J, Sampalis JS, Beaudet F, et al. Adult Still's disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore). 1991 Mar;70(2):118-36. Abstract

93. Kalyoncu U, Kasifoglu T, Omma A, et al. Derivation and validation of adult Still Activity Score (SAS). Joint Bone Spine. 2023 Jan;90(1):105499.Full text  Abstract

94. Hu Q, Wang M, Jia J, et al. Tofacitinib in refractory adult-onset Still's disease: 14 cases from a single centre in China. Ann Rheum Dis. 2020 Jun;79(6):842-4.Full text  Abstract

95. Kacar M, Fitton J, Gough AK, et al. Mixed results with baricitinib in biological-resistant adult-onset Still's disease and undifferentiated systemic autoinflammatory disease. RMD Open. 2020 Jul;6(2):e001246.Full text  Abstract

96. US Food and Drug Administration. FDA requires warnings about increased risk of serious heart-related events, cancer, blood clots and death for JAK inhibitors that treat certain chronic inflammatory conditions. 2021 [internet publication].Full text

97. European Medicines Agency. EMA confirms measures to minimise risk of serious side effects with Janus kinase inhibitors for chronic inflammatory disorders​. 2023 [internet publication].Full text

98. Medicines and Healthcare Products Regulatory Agency. Janus kinase (JAK) inhibitors: new measures to reduce risks of major cardiovascular events, malignancy, venous thromboembolism, serious infections and increased mortality​. 2023 [internet publication].Full text

99. Gabay C, Fautrel B, Rech J, et al. Open-label, multicentre, dose-escalating phase II clinical trial on the safety and efficacy of tadekinig alfa (IL-18BP) in adult-onset Still's disease. Ann Rheum Dis. 2018 Jun;77(6):840-7.Full text  Abstract

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