References

rsp

Научно-практическая ревматология

Rheumatology Science and Practice

1995-44841995-4492

IMA-PRESS, LLC

10.14412/1995-4484-2018-506-514

rsp-2598

Research Article

ОБЗОРЫ

Особенности болезни Стилла, развившейся у взрослых, в XXI в.

CHARACTERISTICS OF ADULT-ONSET STILL’S DISEASE IN THE 21ST CENTURY

Лебедева

В. В.

Lebedeva

V. V.

Муравьев

Ю. В.

Muravyev

Yu. V.

murawyu@mail.ru

ФГБНУ «Научноисследовательский институт ревматологии им. В.А. Насоновой»РоссияV.A. Nasonova Research Institute of Rheumatology, Moscow,Russian Federation

2018

08092018

564506514

2018

Лебедева В.В., Муравьев Ю.В.

Lebedeva V.V., Muravyev Y.V.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://rsp.mediar-press.net/rsp/article/view/2598

В обзоре представлены современные данные о болезни Стилла, развившейся у взрослых: причинах ее возникновения, особенностях патогенеза, диагностики, клинической картины (с выделением субтипов), лечения с учетом характера течения и угрожающих жизни осложнений.

The review gives an update on adult-onset Still’s disease: on its causes, the specific features of its pathogenesis, diagnosis, clinical presentations (with identification of subtypes), and treatment with consideration for the nature of the course of the disease and life-threatening complications.

болезнь Стилларазвившаяся у взрослыхгенно-инженерные биологические препаратыглюкокортикоидынестероидные противовоспалительные препараты

adult-onset Still’s diseasebiological agentsglucocorticoidsnonsteroidal anti-inflammatory drugs

References1

Still GF. On a form of chronic joint disease in children. Med Chir Trans. 1897;80:47-60 (Reprinted in: Arch Dis Child. 1941;16:156-65). doi: 10.1136/adc.16.87.156

Bywaters EG. Still’s disease in the adult. Ann Rheum Dis. 1971;30:121-33. doi: 10.1136/ard.30.2.121

Tanaka S, Matsumoto Y, Ohnishi H, et al. [Comparison of clinical features of childhood and adult onset Still’s disease]. Ryumachi Rheum. 1991;31:511-8.

Uppal SS, Pande IR, Kumar A, et al. Adult onset Still’s disease in northern India: comparison with juvenile onset Still’s disease. Br

J Rheumatol. 1995;34:429-34. doi: 10.1093/rheumatology/34.5.429

Luthi F, Zufferey P, Hofer MF, So AK. «Adolescent-onset Still’s disease»: characteristics and outcome in comparison with adult-onset Still’s disease. Clin Exp Rheumatol. 2002;20:427-30.

Van de Putte LB, Wouters JM. Adult-onset Still’s disease. Baillieres Clin Rheumatol. 1991;5:263-75. doi: 10.1016/S0950-3579(05)80283-3

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.

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. doi: 10.1136/ard.54.7.587

doi: 10.1136/ard.54.7.587

Kaipiainen-Seppä nen O, Aho K. Incidence of rare systemic rheumatic and connective tissue diseases in Finland. J Intern Med. 1996;240:81-4. doi: 10.1046/j.1365-2796.1996.14843000.x

Evensen KJ, Nossent HC. Epidemiology and outcome of adult-onset Still’s disease in Northern Norway. Scand J Rheumatol. 2006;35:48-51. doi: 10.1080/03009740510026616

Joung CI, Lee HS, Lee SW, et al. Association between HLADRB1 and clinical features of adult onset Still’s disease in Korea. Clin Exp Rheumatol. 2003;21:489-92.

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. 2001;40:1398-404. doi: 10.1093/rheumatology/40.12.1398

Sampalis JS, Medsger TA Jr, Fries JF, et al. Risk factors for adult Still’s disease. J Rheumatol. 1996;23:2049-54.

Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis. 2006;65:564-72. doi: 10.1136/ard.2005.042143

Mavragani CP, Spyridakis EG, Koutsilieris M. Adult-onset Still’s disease: from pathophysiology to targeted therapies. Int J Inflam. 2012;2012:879020. doi: 10.1155/2012/879020

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;63:1300-6. doi: 10.1136/ard.2003.013680

Chen DY, Chen YM, Lan JL, et al. Potential role of Th17 cells in the pathogenesis of adult-onset Still’s disease. Rheumatology. 2010;49:2305-12. doi: 10.1093/rheumatology/keq284

Murav'ev YuV, Zhukovskaya GN, Lebedeva VV. About Still's syndrome in adults. Revmatologiya. 1991;(1):37-40 (In Russ.)

Муравьев ЮВ, Жуковская ГН, Лебедева ВВ. О синдроме Стилла у взрослых. Ревматология. 1991;(1):37-40

Murav'ev YuV, Nasonov EL. Adult-onset Still's disease. Nauchno-Prakticheskaya Revmatologiya = Rheumatology Science and Practice. 2011;49(2):58-65 (In Russ.)]. doi: 10.14412/1995-4484-2011-604

Муравьев ЮВ, Насонов ЕЛ. Болезнь Стилла, развившаяся

Baxevanos G, Tzimas T, Pappas G, Akritidis N. A series of 22 patients with adult-onset Still’s disease presenting with fever of unknown origin. A difficult diagnosis? Clin Rheumatol. 2012;31:49-53. doi: 10.1007/s10067-011-1761-y

у взрослых. Научно-практическая ревматология. 2011;49(2):58-65

Chen PD, Yu SL, Chen S, et al. Retrospective study of 61 patients with adult-onset Still’s disease admitted with fever of unknown origin in China. Clin Rheumatol. 2012;31:175-8. doi: 10.1007/s10067-011-1798-y

doi: 10.14412/1995-4484-2011-604

Laskari K, Tzioufas AG, Moutsopoulos HM. Efficacy and longterm follow-up of IL-1R inhibitor anakinra in adults with Still’s disease: a case-series study. Arthritis Res Ther. 2011;13:R91. doi: 10.1186/ar3366

Riera E, Olive A, Narvaez J, et al. Adult onset Still’s disease: review of 41 cases. Clin Exp Rheumatol. 2011;29:331-6.

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;29:1015-9. doi: 10.1007/s10067-010-1516-1

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;28:41-8.

Riera E, Olive A, Narvaez J, et al. Adult onset Still’s disease: review of 41 cases. Clin Exp Rheumatol. 2011;29:331-6.

Lee SW, Park YB, Song JS, Lee SK. The mid-range of the adjusted level of ferritin can predict the chronic course in patients with adult onset Still’s disease. J Rheumatol. 2009;36:156-62.

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;29:1015-9. doi: 10.1007/s10067-010-1516-1

Mitamura M, Tada Y, Koarada S, et al. Cyclosporin A treatment for Japanese patients with severe adult-onset Still’s disease. Mod Rheumatol. 2009;19:57-63. doi: 10.3109/s10165-008-0126-0

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;28:41-8.

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;18:480-5. doi: 10.3109/s10165-008-0104-6

Lee SW, Park YB, Song JS, Lee SK. The mid-range of the adjusted level of ferritin can predict the chronic course in patients with adult onset Still’s disease. J Rheumatol. 2009;36:156-62.

Singh S, Samant R, Joshi VR. Adult onset Still’s disease: a study of 14 cases. Clin Rheumatol. 2008;27:35-9. doi: 10.1007/s10067-007-0648-4

Mitamura M, Tada Y, Koarada S, et al. Cyclosporin A treatment for Japanese patients with severe adult-onset Still’s disease. Mod Rheumatol. 2009;19:57-63. doi: 10.3109/s10165-008-0126-0

Uppal SS, Al-Mutairi M, Hayat S, et al. Ten years of clinical experience with adult onset Still’s disease: is the outcome improving? Clin Rheumatol. 2007;26:1055-60. doi: 10.1007/s10067-006-0440-x

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;18:480-5. doi: 10.3109/s10165-008-0104-6

Pay S, Tü rkcapar 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;25:639-44. doi: 10.1007/s10067-005-0138-5

Singh S, Samant R, Joshi VR. Adult onset Still’s disease: a study of 14 cases. Clin Rheumatol. 2008;27:35-9. doi: 10.1007/s10067-007-0648-4

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

Uppal SS, Al-Mutairi M, Hayat S, et al. Ten years of clinical experience with adult onset Still’s disease: is the outcome improving? Clin Rheumatol. 2007;26:1055-60. doi: 10.1007/s10067-006-0440-x

Pouchout 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. doi: 10.1097/00005792-199103000-00004

Reginato AJ, Schumacher HR Jr, Baker DG, et al. Adult onset Still’s disease: experience in 23 patients and literature review with emphasis on organ failure. Semin Arthritis Rheum. 1987;17:39-57. doi: 10.1016/0049-0172(87)90015-1

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

Bjö rkengren AG, Pathria MN, Sartoris DJ, et al. Carpal alterations in adult-onset Still disease, juvenile chronic arthritis and adult-onset rheumatoid arthritis: comparative study. Radiology. 1987;165:545-8. doi: 10.1148/radiology.165.2.3659381

Appenzeller S, Castro GR, Costallat LT, et al. Adult-onset Still’ disease in southeast Brazil. J Clin Rheumatol. 2005;11:76-80. doi: 10.1097/01.rhu.0000158544.38663.0a

Yamamoto T. Cutaneous manifestations associated with adult-onset Still’s disease: important diagnostic values. Rheumatol Int. 2012;32:2233-7. doi: 10.1007/s00296-011-2330-z

Nguyen KH, Weisman MH. Severe sore throat as a presenting symptom of adult onset Still’s disease: a case series and review of the literature. J Rheumatol. 1997;24:592-7.

Appenzeller S, Castro GR, Costallat LT, et al. Adult-onset Still’ disease in southeast Brazil. J Clin Rheumatol. 2005;11:76-80. doi: 10.1097/01.rhu.0000158544.38663.0a

Min JK, Cho CS, Kim HY, Oh EJ. Bone marrow findings in patients with adult Still’s disease. Scand J Rheumatol. 2003;32:119-21. doi: 10.1080/03009740310000148

Yamamoto T. Cutaneous manifestations associated with adult-onset Still’s disease: important diagnostic values. Rheumatol Int. 2012;32:2233-7. doi: 10.1007/s00296-011-2330-z

Ohta A, Yamaguchi M, Kaneoka H, et al. Adult Still’s disease: review of 228 cases from the literature. J Rheumatol. 1987;14:1139-46.

Nguyen KH, Weisman MH. Severe sore throat as a presenting symptom of adult onset Still’s disease: a case series and review of the literature. J Rheumatol. 1997;24:592-7.

Andres E, Kurts JE, Perrin AE, et al. Retrospective monocentric study of 17 patients with adult Still’s disease with special focus on liver abnormalities. Hepatogastroenterology. 2003;50:192-5.

Min JK, Cho CS, Kim HY, Oh EJ. Bone marrow findings in patients with adult Still’s disease. Scand J Rheumatol. 2003;32:119-21. doi: 10.1080/03009740310000148

Akritidis N, Giannakakis I, Giouglis T, et al. Ferritin levels and response to treatment in patients with Adult Still’s disease. J Rheumatol. 1996;23:201-2.

Ohta A, Yamaguchi M, Kaneoka H, et al. Adult Still’s disease: review of 228 cases from the literature. J Rheumatol. 1987;14:1139-46.

Fautrel B, Le Moёl G, Saint-Marcoux B, et al. Diagnostic value of ferritin and glycosylated ferritin in adult onset Still’s disease. J Rheumatol. 2001;28:3223.

Andres E, Kurts JE, Perrin AE, et al. Retrospective monocentric study of 17 patients with adult Still’s disease with special focus on liver abnormalities. Hepatogastroenterology. 2003;50:192-5.

Cush JJ, Medsger Jr T, Christy WC, et al. Adult-onset Still’s disease: clinical course and outcome. Arthritis Rheum. 1987;30:186-94. doi: 10.1002/art.1780300209

Akritidis N, Giannakakis I, Giouglis T, et al. Ferritin levels and response to treatment in patients with Adult Still’s disease. J Rheumatol. 1996;23:201-2.

Fautrel B, Zing E, Golmard JL, et al. Proposal for a new set ofclassification criteria for adult-onset Still disease. Medicine (Baltimore). 2002;81:194-200. doi: 10.1097/00005792-200205000-00003

Fautrel B, Le Moёl G, Saint-Marcoux B, et al. Diagnostic value of ferritin and glycosylated ferritin in adult onset Still’s disease. J Rheumatol. 2001;28:3223.

Masson C, Le Loet X, Liote F, et al. Comparative study of 6 types of criteria in adult Still’s disease. J Rheumatol. 1996;23:495-7.

Cush JJ, Medsger Jr T, Christy WC, et al. Adult-onset Still’s disease: clinical course and outcome. Arthritis Rheum. 1987;30:186-94. doi: 10.1002/art.1780300209

Wouters JM, van de Putte LB. Adult-onset Still’s disease; clinical and laboratory features, treatment and progress of 45 cases. Q J Med. 1986;61:1055-65.

Fautrel B, Zing E, Golmard JL, et al. Proposal for a new set ofclassification criteria for adult-onset Still disease. Medicine (Baltimore). 2002;81:194-200. doi: 10.1097/00005792-200205000-00003

McGonagle D, McDermott MF. A proposed classification of the immunological diseases. PLoS Med. 2006;3:e297. doi: 10.1371/journal.pmed.0030297

Masson C, Le Loet X, Liote F, et al. Comparative study of 6 types of criteria in adult Still’s disease. J Rheumatol. 1996;23:495-7.

Kastner DL, Aksentijevich I, Goldbach-Mansky R. Autoinflammatory disease reloaded: a clinical perspective. Cell. 2010;140:784-90. doi: 10.1016/j.cell.2010.03.002

Wouters JM, van de Putte LB. Adult-onset Still’s disease; clinical and laboratory features, treatment and progress of 45 cases. Q J Med. 1986;61:1055-65.

Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Seve P. Adult-onset Still’s disease. Autoimmun Rev. 2014;13:708-22. doi: 10.1016/j.autrev.2014.01.058

McGonagle D, McDermott MF. A proposed classification of the immunological diseases. PLoS Med. 2006;3:e297.

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. doi: 10.1016/j.autrev.2014.08.032

doi: 10.1371/journal.pmed.0030297

Canna SW. Editorial. Interferon-: friend or foe in systemic juvenile idiopathic arthritis and adult Still’s disease. Arthritis Rheum. 2014;66:1072-6. doi: 10.1002/art.38362

Kastner DL, Aksentijevich I, Goldbach-Mansky R. Autoinflammatory disease reloaded: a clinical perspective. Cell. 2010;140:784-90. doi: 10.1016/j.cell.2010.03.002

Shimizu M, Nakagishi Y, Yachie A. Distinct subsets of patients with systemic juvenile idiopathic arthritis based on their cytokine profiles. Cytokine. 2013;61:345-8. doi: 10.1016/j.cyto.2012.11.025

Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Seve P. Adult-onset Still’s disease. Autoimmun Rev. 2014;13:708-22.

Chen D-Y, Lan J-L, Lin F-J, Hsieh T-Y. Proinflammatory cytokine profiles in sera and pathological tissues of patients with active untreated adult onset Still’s disease. J Rheumatol. 2004;31:2189-98.

doi: 10.1016/j.autrev.2014.01.058

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 Oct 7. [Epub ahead of print].

Shimizu M, Yokoyama T, Yamada K, et al. Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis. Rheumatology. 2010;49:1645-53. doi: 10.1093/rheumatology/keq133

Canna SW. Editorial. Interferon-: friend or foe in systemic juvenile idiopathic arthritis and adult Still’s disease. Arthritis Rheum. 2014;66:1072-6. doi: 10.1002/art.38362

Gerfaud-Valentin M, Maucort-Boulch D, Hot A, et al. Adult-onset Still disease: manifestations, treatments, outcome, and prognostic factors in 57 patients. Medicine (Baltimore). 2014;93:91-9. doi: 10.1097/MD.0000000000000021

Shimizu M, Nakagishi Y, Yachie A. Distinct subsets of patients with systemic juvenile idiopathic arthritis based on their cytokine profiles. Cytokine. 2013;61:345-8. doi: 10.1016/j.cyto.2012.11.025

Franchini S, Dagna L, Salvo F, et al. Efficacy of traditional and biologic agents in different clinical phenotypes of adult-onset Still’s disease. Arthritis Rheum. 2010;62:2530-5. doi: 10.1002/art.27532

Kim YJ, Koo BS, Kim Y-G, et al. Clinical features and prognosis in 82 patients with adult-onset Still’s disease. Clin Exp Rheumatol. 2014;32:28-33.

Hot A, Toh M-L, Coppere B, et al. Reactive hemophagocytic syndrome in adult-onset Still disease: clinical features and longterm outcome: a case-control study of 8 patients. Medicine (Baltimore). 2010;89:37-46. doi: 10.1097/MD.0b013e3181caf100

Nagashima T, Iwamoto M, Matsumoto K, Minota S. Interleukin-18 in adult-onset Still’s disease: treatment target or disease activity indicator? Intern Med. 2012;51:449. doi: 10.2169/internalmedicine.51.6678

Kim H-A, Sung J-M, Suh C-H. Therapeutic responses and prognosis in adult-onset Still’s disease. Rheumatol Int. 2012;32:1291-8. doi: 10.1007/s00296-011-1801-6

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

Kim YJ, Koo BS, Kim Y-G, et al. Clinical features and prognosis in 82 patients with adult-onset Still’s disease. Clin Exp Rheumatol. 2014;32:28-33.

Vignes S, Wechsler B, Amoura Z, et al. Intravenous immunoglobulin in adult Still’s disease refractory to non-steroidal anti-inflammatory drugs. Clin Exp Rheumatol. 1998;16:295-8.

Permal S, Wechsler B, Cabane J, et al. [Treatment of Still’s disease in adults with intravenous immunoglobulins]. Rev Med Interne. 1995;16:250-4. doi: 10.1016/0248-8663(96)80703-X

Nagashima T, Iwamoto M, Matsumoto K, Minota S. Interleukin-18 in adult-onset Still’s disease: treatment target or disease activity indicator? Intern Med. 2012;51:449.

Murav'ev YuV. The use of genetically engineered biological agents in the disease of Still, developed in adults. In: Nasonov EL, editor. Genno-inzhenernye biologicheskie preparaty v lechenii revmatoidnogo artrita [Genetically engineered biological agents in the treatment of rheumatoid arthritis]. Moscow: IMA-PRESS; 2013. P. 398-404 (In Russ.)

doi: 10.2169/internalmedicine.51.6678

Benucci M, Li GF, Del Rosso A, Manfredi M. Adalimumab (anti-TNF-alpha) therapy to improve the clinical course of adult-onset Still’s disease: the first case report. Clin Exp Rheumatol. 2005;23:733.

Kim H-A, Sung J-M, Suh C-H. Therapeutic responses and prognosis in adult-onset Still’s disease. Rheumatol Int. 2012;32:1291-8. doi: 10.1007/s00296-011-1801-6

Felson DT, Anderson JJ, Boers M, et al. American College of Rheumatology. Preliminary definition of improvement in rheumatoid arthritis. Arthritis Rheum. 1995;38:727-35.

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

doi: 10.1002/art.1780380602

Vignes S, Wechsler B, Amoura Z, et al. Intravenous immunoglobulin in adult Still’s disease refractory to non-steroidal anti-inflammatory drugs. Clin Exp Rheumatol. 1998;16:295-8.

Husni ME, Maier AL, Mease PJ, et al. Etanercept in the treatment of adult patients with Still’s disease. Arthritis Rheum. 2002;46:1171-6. doi: 10.1002/art.10231

Permal S, Wechsler B, Cabane J, et al. [Treatment of Still’s disease in adults with intravenous immunoglobulins]. Rev Med Interne. 1995;16:250-4. doi: 10.1016/0248-8663(96)80703-X

Cavagna L, Caporali R, Epis O, et al. Infliximab in the treatment of adult Still’s disease refractory to conventional therapy. Clin Exp Rheumatol. 2001;19:329-32.

Муравьев ЮВ. Применение генно-инженерных биологиче-ских препаратов при болезни Стилла, развившейся у взрос-лых. В кн.: Насонов ЕЛ, редактор. Генно-инженерные биоло-гические препараты в лечении ревматоидного артрита. Моск-ва: ИМА-ПРЕСС; 2013. С. 398-404

Kraetsch HG, Antoni C, Kalden JR, Manger B. Successful treatment of a small cohort of patients with adult onset of Still’s disease with infliximab: first experiences. Ann Rheum Dis. 2001;60 Suppl 3:iii55-7.

Benucci M, Li GF, Del Rosso A, Manfredi M. Adalimumab (anti-TNF-alpha) therapy to improve the clinical course of adult-onset Still’s disease: the first case report. Clin Exp Rheumatol. 2005;23:733.

Kokkinos A, Iliopoulos A, Greka P, et al. Successful treatment of refractory adult-onset Still’s disease with infliximab. A prospective, non-comparative series of four patients. Clin Rheumatol. 2004;23:45-9. doi: 10.1007/s10067-003-0775-5

Felson DT, Anderson JJ, Boers M, et al. American College of Rheumatology. Preliminary definition of improvement in rheumatoid arthritis. Arthritis Rheum. 1995;38:727-35.

Fautrel B, Sibilia J, Mariette X, Combe B; Club Rhumatismes et Inflammation. Tumour necrosis factor alpha blocking agents in refractory adult Still’s disease: an observational study of 20 cases. Ann Rheum Dis. 2005;64:262-6. doi: 10.1136/ard.2004.024026

doi: 10.1002/art.1780380602

Aikawa NE, Ribeiro AC, Saad CG, et al. Is anti-TNF switching in refractory Still’s disease safe and effective? Clin Rheumatol. 2011;30:1129-34. doi: 10.1007/s10067-011-1735-0

Husni ME, Maier AL, Mease PJ, et al. Etanercept in the treatment of adult patients with Still’s disease. Arthritis Rheum. 2002;46:1171-6. doi: 10.1002/art.10231

Kaneko K, Kaburaki M, Muraoka S, et al. Exacerbation of adult-onset Still’s disease, possibly related to elevation of serum tumor necrosis factor-alpha after etanercept administration. Int J Rheum Dis. 2010;13:e67-9. doi: 10.1111/j.1756-185X.2010.01544.x

Cavagna L, Caporali R, Epis O, et al. Infliximab in the treatment of adult Still’s disease refractory to conventional therapy. Clin Exp Rheumatol. 2001;19:329-32.

Vasques Godinho FM, Parreira Santos MJ, Canas da Silva J. Refractory adult onset Still’s disease successfully treated with anakinra. Ann Rheum Dis. 2005;64:647-8.

doi: 10.1136/ard.2004.026617

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. doi: 10.1002/art.21061

Kalliolias GD, Georgiou PE, Antonopoulos IA, et al. Anakinra treatment in patients with adult-onset Still’s disease is fast, effective, safe and steroid sparing: experience from an uncontrolled trial. Ann Rheum Dis. 2007;66:842-3. doi: 10.1136/ard.2006.066381

Aikawa NE, Ribeiro AC, Saad CG, et al. Is anti-TNF switching in refractory Still’s disease safe and effective? Clin Rheumatol. 2011;30:1129-34. doi: 10.1007/s10067-011-1735-0

Kö tter I, Wacker A, Koch S, et al. Anakinra in patients with treatment-resistant adult-onset Still’s disease: four case reports with serial cytokine measurements and a review of the literature. Semin Arthritis Rheum. 2007;37:189-97. doi: 10.1016/j.semarthrit.2007.04.002

Rudinskaya A, Trock DH. Successful treatment of a patient with refractory adult-onset Still disease with anakinra. J Clin Rheumatol. 2003;9:330-2. doi: 10.1097/01.rhu.0000089966.48691.7f

Vasques Godinho FM, Parreira Santos MJ, Canas da Silva J. Refractory adult onset Still’s disease successfully treated with anakinra. Ann Rheum Dis. 2005;64:647-8. doi: 10.1136/ard.2004.026617

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;81:376-7. doi: 10.1016/j.jbspin.2013.12.011

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. doi: 10.1002/art.21061

Kontzias A, Efthimiou P. The use of canakinumab, a novel IL-1long-acting inhibitor, in refractory adult-onset Still’s disease. Semin Arthritis Rheum. 2012;42:201-5. doi: 10.1016/j.semarthrit.2012.03.004

Ruperto N, Quartier P, Wulffraat N, et al. A phase II, multicenter, open-label study evaluating dosing and preliminary safety and efficacy of canakinumab in systemic juvenile idiopathic arthritis with active systemic features. Arthritis Rheum. 2012;64:557-67. doi: 10.1002/art.33342

Ruperto N, Brunner HI, Quartier P, et al. Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012;367:2396-406. doi: 10.1056/NEJMoa1205099

Rudinskaya A, Trock DH. Successful treatment of a patient with refractory adult-onset Still disease with anakinra. J Clin Rheumatol. 2003;9:330-2. doi: 10.1097/01.rhu.0000089966.48691.7f

Puechal X, DeBandt M, Berthelot J-M, et al. Tocilizumab in refractory adult Still’s disease. Arthritis Care Res. 2011;63:155-9. doi: 10.1002/acr.20319

Cipriani P, Ruscitti P, Carubbi F, et al. Tocilizumab for the treatment of adult-onset Still’s disease: results from a case series. Clin Rheumatol. 2014;33:49-55. doi: 10.1007/s10067-013-2381-5

Elkayam O, Jiries N, Dranitzki Z, et al. Tocilizumab in adult-onset Still’s disease: the Israeli experience. J Rheumatol. 2014;41:244-7. doi: 10.3899/jrheum.130881

Ortiz-Sanjuan F, Blanco R, Calvo-Rio V, et al. Efficacy of tocilizumab in conventional treatment-refractory adult-onset Still’s disease: multicenter retrospective open-label study of thirty-four patients. Arthritis Rheum. 2014;66:1659-65. doi: 10.1002/art.38398

Ruperto N, Brunner HI, Quartier P, et al. Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012;367:2396-406. doi: 10.1056/NEJMoa1205099

Ahmadi-Simab K, Lamprecht P, Jankowiak C, Gross WL. Successful treatment of refractory adult onset Still's disease with rituximab. Ann Rheum Dis. 2006;65:1117-8. doi: 10.1136/ard.2005.047621

Puechal X, DeBandt M, Berthelot J-M, et al. Tocilizumab in refractory adult Still’s disease. Arthritis Care Res. 2011;63:155-9. doi: 10.1002/acr.20319

Arlet J-B, Le TH, Marinho A, et al. Reactive haemophagocytic syndrome in adult-onset Still’s disease: a report of six patients and a review of the literature. Ann Rheum Dis. 2006;65:1596-601. doi: 10.1136/ard.2005.046904

Cipriani P, Ruscitti P, Carubbi F, et al. Tocilizumab for the treatment of adult-onset Still’s disease: results from a case series. Clin Rheumatol. 2014;33:49-55. doi: 10.1007/s10067-013-2381-5

Ravelli A, Grom AA, Behrens EM, Cron RQ. Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment. Genes Immun. 2012;13:289-98. doi: 10.1038/gene.2012.3

Elkayam O, Jiries N, Dranitzki Z, et al. Tocilizumab in adult-onset Still’s disease: the Israeli experience. J Rheumatol. 2014;41:244-7. doi: 10.3899/jrheum.130881

Silva CA, Silva CH, Robazzi TC, et al. [Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis]. J Pediatr (Rio J). 2004;80:517-22. doi: 10.2223/1267

Ramanan AV, Schneider R. Macrophage activation syndrome following initiation of etanercept in a child with systemic onset juvenile rheumatoid arthritis. J Rheumatol. 2003;30:401-3.

Ahmadi-Simab K, Lamprecht P, Jankowiak C, Gross WL. Successful treatment of refractory adult onset Still's disease with rituximab. Ann Rheum Dis. 2006;65:1117-8. doi: 10.1136/ard.2005.047621

Tristano AG. Macrophage activation syndrome: a frequent but under-diagnosed complication associated with rheumatic diseases. Med Sci Monit. 2008;14:RA27-36.

Arlet J-B, Le TH, Marinho A, et al. Reactive haemophagocytic syndrome in adult-onset Still’s disease: a report of six patients and a review of the literature. Ann Rheum Dis. 2006;65:1596-601.

Efthimiou P, Kadavath S, Mehta B. Life-threatening complications of adult-onset Still’s disease. Clin Rheumatol. 2014;33:305-14. doi: 10.1007/s10067-014-2487-4

doi: 10.1136/ard.2005.046904

Mizrahi M, Ben-Chetrit E. Relapsing macrophage activating syndrome in a 15-year-old girl with Still’s disease: a case report.J Med Case Rep. 2009;3:138. doi: 10.1186/1752-1947-3-138

Kelly A, Ramanan AV. A case of macrophage activation syndrome successfully treated with anakinra. Nat Clin Pract Rheumatol. 2008;4:615-20. doi: 10.1038/ncprheum0919

100

Silva CA, Silva CH, Robazzi TC, et al. [Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis]. J Pediatr (Rio J). 2004;80:517-22. doi: 10.2223/1267

Bruck N, Suttorp M, Kabus M, et al. Rapid and sustained remission of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome through treatment with anakinra and corticosteroids. J Clin Rheumatol. 2011;17:23-7.

101

Ramanan AV, Schneider R. Macrophage activation syndrome following initiation of etanercept in a child with systemic onset juvenile rheumatoid arthritis. J Rheumatol. 2003;30:401-3.

doi: 10.1097/RHU.0b013e318205092d

102

Tristano AG. Macrophage activation syndrome: a frequent but under-diagnosed complication associated with rheumatic diseases. Med Sci Monit. 2008;14:RA27-36.

Record JL, Beukelman T, Cron RQ. Combination therapy of abatacept and anakinra in children with refractory systemic juvenile idiopathic arthritis: a retrospective case series. J Rheumatol. 2011;38:180-1. doi: 10.3899/jrheum.100726

103

Efthimiou P, Kadavath S, Mehta B. Life-threatening complications of adult-onset Still’s disease. Clin Rheumatol. 2014;33:305-14. doi: 10.1007/s10067-014-2487-4

Pamuk ON, Pamuk GE, Usta U, Cakir N. Hemophagocytic syndrome in one patient with adult-onset Still’s disease. Presentation with febrile neutropenia. Clin Rheumatol. 2007;26:797-800. doi: 10.1007/s10067-006-0238-x

104

Mizrahi M, Ben-Chetrit E. Relapsing macrophage activating syndrome in a 15-year-old girl with Still’s disease: a case report.J Med Case Rep. 2009;3:138. doi: 10.1186/1752-1947-3-138

Stern A, Riley R, Buckley L. Worsening of macrophage activation syndrome in a patient with adult onset Still’s disease after initiation of etanercept therapy. J Clin Rheumatol. 2001;7:252-6. doi: 10.1097/00124743-200108000-00013

105

Kelly A, Ramanan AV. A case of macrophage activation syndrome successfully treated with anakinra. Nat Clin Pract Rheumatol. 2008;4:615-20. doi: 10.1038/ncprheum0919

Kobayashi M, Takahashi Y, Yamashita H, et al. Benefit and a possible risk of tocilizumab therapy for adult-onset Still’s disease accompanied by macrophage-activation syndrome. Mod Rheumatol. 2011;21:92-6. doi: 10.3109/s10165-010-0348-9

106

Aellen P, Raccaud O, Waldburger M, et al. [Still’s disease in adults with disseminated intravascular coagulation]. Schweiz Rundsch Med Prax. 1991;80:376-8.

107

doi: 10.1097/RHU.0b013e318205092d

Arai Y, Handa T, Mitani K. [Adult-onset Still disease presenting with disseminated intravascular coagulation]. Rinsho Ketsueki. 2004;45:316-8.

108

Рark J-H, Bae JH, Choi Y-S, et al. Adult-onset Still’s disease with disseminated intravascular coagulation and multiple organ dysfunctions dramatically treated with cyclosporine A. J Korean Med Sci. 2004;19:137-41. doi: 10.3346/jkms.2004.19.1.137

109

Pamuk ON, Pamuk GE, Usta U, Cakir N. Hemophagocytic syndrome in one patient with adult-onset Still’s disease. Presentation with febrile neutropenia. Clin Rheumatol. 2007;26:797-800.

Yokoyama M, Suwa A, Shinozawa T, et al. [A case of adult onsetStill’s disease complicated with adult respiratory distress syndrome and disseminated intravascular coagulation]. Nihon Rinsho Meneki Gakkai Kaishi. 1995;18:207-14. doi: 10.2177/jsci.18.207

110

doi: 10.1007/s10067-006-0238-x

Mori T, Tanigawa M, Iwasaki E, et al. [Cyclosporine therapy of adult onset Still’s disease with disseminated intravascular coagulation]. Nihon Rinsho Meneki Gakkai Kaishi. 1993;34:147-52.

111

Matsumoto K, Nagashima T, Takatori S, et al. Glucocorticoid and cyclosporine refractory adult onset Still’s disease successfully treated with tocilizumab. Clin Rheumatol. 2009;28:485-7. doi: 10.1007/s10067-009-1097-z

112

Gerfaud-Valentin M, Seve P, Iwaz J, et al. Myocarditis in adult-onset Still disease. Medicine (Baltimore). 2014;93:280-9. doi: 10.1097/MD.0000000000000112

113

Aellen P, Raccaud O, Waldburger M, et al. [Still’s disease in adults with disseminated intravascular coagulation]. Schweiz Rundsch Med Prax. 1991;80:376-8.

Perez MG, Rodwig FR Jr. Chronic relapsing thrombotic thrombocytopenic purpura in adult onset Still’s disease. South Med J. 2003;96:46-9. doi:10.1097/01.SMJ.0000047763.56451.F0

114

Arai Y, Handa T, Mitani K. [Adult-onset Still disease presenting with disseminated intravascular coagulation]. Rinsho Ketsueki. 2004;45:316-8.

Masuyama A, Kobayashi H, Kobayashi Y, et al. A case of adult-onset Still’s disease complicated by thrombotic thrombocytopenic purpura with retinal microangiopathy and rapidly fatal cerebral edema. Mod Rheumatol. 2013;23:379-85. doi: 10.3109/s10165-012-0650-9

115

Sayarlioglu M, Sayarlioglu H, Ozkaya M, et al. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome and adult onset Still’s disease: case report and review of the literature. Mod Rheumatol. 2008;18,403-6. doi: 10.3109/s10165-008-0061-0

116

Yokoyama M, Suwa A, Shinozawa T, et al. [A case of adult onset

Onundarson PT, Rowe JM, Heal JM, Francis CW. Response to plasma exchange and splenectomy in thrombotic thrombocytopenic purpura. A 10-year experience at a single institution. Arch Intern Med. 1992;152:791-6. doi: 10.1001/archinte.1992.00400160089017

117

Still’s disease complicated with adult respiratory distress syndrome and disseminated intravascular coagulation]. Nihon Rinsho Meneki Gakkai Kaishi. 1995;18:207-14. doi: 10.2177/jsci.18.207

Cheema GS, Quismorio FP. Pulmonary involvement in adult-onset Still’s disease. Curr Opin Pulm Med. 1999;5:305-9. doi: 10.1097/00063198-199909000-00007

118

Mori T, Tanigawa M, Iwasaki E, et al. [Cyclosporine therapy of adult onset Still’s disease with disseminated intravascular coagulation]. Nihon Rinsho Meneki Gakkai Kaishi. 1993;34:147-52.

Sari I, Birlik M, Binicier O, et al. A case of adult-onset Still’s disease complicated with diffuse alveolar hemorrhage. J Korean Med Sci. 2009;24:155-7. doi: 10.3346/jkms.2009.24.1.155

119

Matsumoto K, Nagashima T, Takatori S, et al. Glucocorticoid and cyclosporine refractory adult onset Still’s disease successfully treated with tocilizumab. Clin Rheumatol. 2009;28:485-7.

Gerfaud-Valentin M, Hot A, Huissoud C, et al. Adult-onsetStill’s disease and pregnancy: about ten cases and review of the literature. Rheumatol Int. 2014;34:867-71. doi: 10.1007/s00296-013-2765-5

120

doi: 10.1007/s10067-009-1097-z

Le Loёt X, Daragon A, Duval C, et al. Adult onset Still’s disease and pregnancy. J Rheumatol. 1993;20:1158-61.

121

Gerfaud-Valentin M, Seve P, Iwaz J, et al. Myocarditis in adult-onset Still disease. Medicine (Baltimore). 2014;93:280-9. doi: 10.1097/MD.0000000000000112

Lockshin MD, Sammaritano LR. Corticosteroids during pregnancy. Scand J Rheumatol. 1998;107 Suppl:136-8. doi: 10.1080/03009742.1998.11720789

122

Perez MG, Rodwig FR Jr. Chronic relapsing thrombotic thrombocytopenic purpura in adult onset Still’s disease. South Med J. 2003;96:46-9. doi: 10.1097/01.SMJ.0000047763.56451.F0

Fischer-Betz R, Specker C, Schneider M. Successful outcome of two pregnancies in patients with adult-onset Still’s disease treated with IL-1 receptor antagonist (anakinra). Clin Exp Rheumatol. 2011;29:1021-3.

123

Berger CT, Recher M, Steiner U, Hauser TM. A patient’s wish: anakinra in pregnancy. Ann Rheum Dis. 2009;68:1794-5. doi: 10.1136/ard.2008.105833

124

125

126

Cheema GS, Quismorio FP. Pulmonary involvement in adult-onset Still’s disease. Curr Opin Pulm Med. 1999;5:305-9.

127

doi: 10.1097/00063198-199909000-00007

128

Sari I, Birlik M, Binicier O, et al. A case of adult-onset Still’s disease complicated with diffuse alveolar hemorrhage. J Korean Med Sci. 2009;24:155-7. doi: 10.3346/jkms.2009.24.1.155

129

Gerfaud-Valentin M, Hot A, Huissoud C, et al. Adult-onsetStill’s disease and pregnancy: about ten cases and review of the literature. Rheumatol Int. 2014;34:867-71. doi: 10.1007/s00296-013-2765-5

130

Le Loёt X, Daragon A, Duval C, et al. Adult onset Still’s disease and pregnancy. J Rheumatol. 1993;20:1158-61.

131

Lockshin MD, Sammaritano LR. Corticosteroids during pregnancy. Scand J Rheumatol. 1998;107 Suppl:136-8.

132

doi: 10.1080/03009742.1998.11720789

133

134

Berger CT, Recher M, Steiner U, Hauser TM. A patient’s wish: anakinra in pregnancy. Ann Rheum Dis. 2009;68:1794-5. doi: 10.1136/ard.2008.105833

The authors declare that there are no conflicts of interest present.