Mimura T, Kondo Y, Ohta A, Iwamoto M, Ota A, Okamoto N, et al. Evidence-based clinical practice guideline for adult Still’s disease. Mod Rheumatol. 2018;28(5):736–57.

Article  PubMed  Google Scholar 

Mitrovic S, Fautrel B. Clinical phenotypes of adult-onset Still’s disease: New insights from pathophysiology and literature findings. J Clin Med. 2021;10(12):2633.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still’s disease. Nat Rev Rheumatol. 2018;14(10):603–18.

Article  PubMed  PubMed Central  Google Scholar 

Castañeda S, Blanco R, González-Gay MA. Adult-onset Still’s disease: Advances in the treatment. Best Pract Res Clin Rheumatol. 2016;30(2):222–38.

Article  PubMed  Google Scholar 

Zhou S, Qiao J, Bai J, Wu Y, Fang H. Biological therapy of traditional therapy-resistant adult-onset Still’s disease: an evidence-based review. Ther Clin Risk Manag. 2018;14:167–71.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Ruscitti P, Ursini F, Cipriani P, De Sarro G, Giacomelli R. Biologic drugs in adult-onset Still’s disease: a systematic review and meta-analysis of observational studies. Expert Rev Clin Immunol. 2017;13(11):1089–97.

Article  CAS  PubMed  Google Scholar 

Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still’s disease. J Autoimmun. 2018;93:24–36.

Article  PubMed  Google Scholar 

Yamabe T, Ohmura SI, Uehara K, Naniwa T. Macrophage activation syndrome in patients with adult-onset Still’s disease under tocilizumab treatment: a single-center observational study. Mod Rheumatol. 2022;32(1):169–76.

Article  PubMed  Google Scholar 

Shimizu M, Nakagishi Y, Kasai K, Yamasaki Y, Miyoshi M, Takei S, et al. Tocilizumab masks the clinical symptoms of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome: the diagnostic significance of interleukin-18 and interleukin-6. Cytokine. 2012;58(2):287–94.

Article  CAS  PubMed  Google Scholar 

Kobayashi M, Takahashi Y, Yamashita H, Kaneko H, Mimori A. Benefit and a possible risk of tocilizumab therapy for adult-onset Still’s disease accompanied by macrophage-activation syndrome. Mod Rheumatol. 2011;21(1):92–6.

Article  PubMed  Google Scholar 

Vercruysse F, Barnetche T, Lazaro E, Shipley E, Lifermann F, Balageas A, et al. Adult-onset Still’s disease biological treatment strategy may depend on the phenotypic dichotomy. Arthritis Res Ther. 2019;21(1):53.

Article  PubMed  PubMed Central  Google Scholar 

Yokota S, Itoh Y, Morio T, Sumitomo N, Daimaru K, Minota S. Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis under treatment with tocilizumab. J Rheumatol. 2015;42(4):712–22.

Article  CAS  PubMed  Google Scholar 

Schulert GS, Minoia F, Bohnsack J, Cron RQ, Hashad S, KonÉ-Paut I, et al. Effect of biologic therapy on clinical and laboratory features of macrophage activation syndrome associated with systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken). 2018;70(3):409–19.

Article  CAS  PubMed  Google Scholar 

Kedor C, Tomaras S, Baeumer D, Feist E. Update on the therapy of adult-onset Still’s disease with a focus on IL-1-inhibition: a systematic review. Ther Adv Musculoskelet Dis. 2021;13:1759720x211059598.

Article  CAS  PubMed  PubMed Central  Google Scholar 

de Boysson H, Février J, Nicolle A, Auzary C, Geffray L. Tocilizumab in the treatment of the adult-onset Still’s disease: current clinical evidence. Clin Rheumatol. 2013;32(1):141–7.

Article  PubMed  Google Scholar 

Naniwa T, Yamabe T, Ohmura SI, Uehara K, Tamechika SY, Maeda S, et al. Baseline clinical features predicting macrophage activation syndrome in patients with systemic adult-onset Still’s disease receiving interleukin-6 inhibitor treatment. Int J Rheum Dis. 2022;25(9):1003–12.

Article  CAS  PubMed  Google Scholar 

Stroup DF, Berlin JA, Morton SC, Olkin I, Williamson GD, Rennie D, et al. Meta-analysis of observational studies in epidemiology: a proposal for reporting. Meta-analysis of Observational Studies in Epidemiology (MOOSE) group. JAMA. 2000;283(15):2008–12.

Article  CAS  PubMed  Google Scholar 

Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19(3):424–30.

CAS  PubMed  Google Scholar 

Agresti A, Coull BA. Approximate is better than “exact” for interval estimation of binomial proportions. Am Stat. 1998;52(2):119–26.

Google Scholar 

Stang A. Critical evaluation of the Newcastle–Ottawa Scale for the assessment of the quality of nonrandomized studies in meta-analyses. Eur J Epidemiol. 2010;25(9):603–5.

Article  PubMed  Google Scholar 

Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, et al. 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol. 2016;68(3):566–76.

Article  PubMed  Google Scholar 

Sota J, Rigante D, Ruscitti P, Insalaco A, Sfriso P, de Vita S, et al. Anakinra drug retention rate and predictive factors of long-term response in systemic juvenile idiopathic arthritis and adult-onset Still disease. Front Pharmacol. 2019;10:918.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Vitale A, Insalaco A, Sfriso P, Lopalco G, Emmi G, Cattalini M, et al. A snapshot on the on-label and off-label use of the interleukin-1 inhibitors in Italy among rheumatologists and pediatric rheumatologists: A nationwide multi-center retrospective observational study. Front Pharmacol. 2016;7:380.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Vitale A, Cavalli G, Colafrancesco S, Priori R, Valesini G, Argolini LM, et al. Long-term retention rate of anakinra in adult onset Still’s disease and predictive factors for treatment response. Front Pharmacol. 2019;10:296.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Rossi-Semerano L, Fautrel B, Wendling D, Hachulla E, Galeotti C, Semerano L, et al. Tolerance and efficacy of off-label anti-interleukin-1 treatments in France: a nationwide survey. Orphanet J Rare Dis. 2015;10:19.

Article  PubMed  PubMed Central  Google Scholar 

Lequerré T, Quartier P, Rosellini D, Alaoui F, De Bandt M, Mejjad O, 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.

Article  PubMed  Google Scholar 

Tang KT, Hsieh CW, Chen HH, Chen YM, Chang SH, Huang PH, et al. The effectiveness of tocilizumab in treating refractory adult-onset Still’s disease with dichotomous phenotypes: IL-18 is a potential predictor of therapeutic response. Clin Rheumatol. 2022;41(2):557–66.

Article  PubMed  Google Scholar 

Kır S, Özgen M, Zontul S. Adult-onset still’s disease and treatment results with tocilizumab. Int J Clin Pract. 2021;75(3): e13936.

Article  PubMed  Google Scholar 

Li T, Gu L, Wang X, Guo L, Shi H, Yang C, et al. A pilot study on tocilizumab for treating refractory adult-onset Still’s disease. Sci Rep. 2017;7(1):13477.

Article  PubMed  PubMed Central  Google Scholar 

Bannai E, Yamashita H, Kaneko S, Ueda Y, Ozaki T, Tsuchiya H, et al. Successful tocilizumab therapy in seven patients with refractory adult-onset Still’s disease. Mod Rheumatol. 2016;26(2):297–301.

Article  PubMed  Google Scholar 

Elkayam O, Jiries N, Dranitzki Z, Kivity S, Lidar M, Levy O, et al. Tocilizumab in adult-onset Still’s disease: the Israeli experience. J Rheumatol. 2014;41(2):244–7.

Article  CAS  PubMed  Google Scholar 

Shakoory B, Geerlinks A, Wilejto M, Kernan K, Hines M, Romano M, et al. The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS). Ann Rheum Dis. 2023. https://doi.org/10.1136/ard-2023-224123. (Online ahead of print).

Article  PubMed  Google Scholar 

Bindoli S, Galozzi P, Doria A, Sfriso P. Intravenous anakinra to curb cytokine storm in adult-onset Still’s disease and in macrophage activation syndrome: a case series. Joint Bone Spine. 2023;90(2): 105524.

Article  CAS  PubMed  Google Scholar 

Kiyonaga Y, Maeshima K, Imada C, Haranaka M, Ishii K, Shibata H. Steroid-sparing effects of etanercept in a patient with steroid-dependent adult-onset Still’s disease. Intern Med. 2014;53(11):1209–13.

Article  PubMed  Google Scholar 

Fautrel B, Sibilia J, Mariette X, Combe B. Tumour necrosis factor alpha blocking agents in refractory adult Still’s disease: an observational study of 20 cases. Ann Rheum Dis. 2005;64(2):262–6.

Article  CAS  PubMed  Google Scholar 

Jia J, Wang M, Meng J, Ma Y, Wang Y, Miao N, et al. Ferritin triggers neutrophil extracellular trap-mediated cytokine storm through Msr1 contributing to adult-onset Still’s disease pathogenesis. Nat Commun. 2022;13(1):6804.

Article  CAS  PubMed  PubMed Central