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Tofacitinib in refractory adult-onset Still’s disease: 14 cases from a single centre in China
  1. Qiongyi Hu,
  2. Mengyan Wang,
  3. Jinchao Jia,
  4. Jialin Teng,
  5. Huihui Chi,
  6. Tingting Liu,
  7. Hong-lei Liu,
  8. Xiaobing Cheng,
  9. Junna Ye,
  10. Yutong Su,
  11. Yue Sun,
  12. Zhuochao Zhou,
  13. Liyan Wan,
  14. Zhihong Wang,
  15. Fan Wang,
  16. Hui Shi,
  17. Chengde Yang
  1. Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
  1. Correspondence to Chengde Yang, Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China; yangchengde{at}sina.com; Dr Hui Shi; shihui_sjtu{at}sina.com

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Adult-onset Still’s disease (AOSD) is an autoinflammatory disease characterised by spiking fever, rash, polyarthralgia, sore throat and even life-threatening complications, such as macrophage activation syndrome and fulminant hepatitis. Excessive and inappropriate production of cytokines is a cornerstone in AOSD pathogenesis.1 Unlike anakinra and tocilizumab, Janus kinases (JAK) inhibitors block the proinflammatory effect of a wide range of cytokines. This range of activity could be beneficial in AOSD patients who are refractory to or intolerant of treatment with biologicals. Anti-interleukin 1 (IL-1) agents are not available in mainland China. Tofacitinib, a JAK1/3 inhibitor, has been proven efficacious in several inflammatory diseases, such as rheumatoid arthritis, systemic lupus erythematosus and psoriasis arthritis.2 To our interest, a case report observed that tofacitinib could ameliorate arthritis in a 13-year-old girl with recalcitrant systemic juvenile idiopathic arthritis,3 which is the juvenile counterpart of AOSD.4 Moreover, a JAK1/2 inhibitor, baricitinib has been reported effective in a 43-year-old patient with refractory AOSD.5 Therefore, JAK inhibitors may be a novel therapeutic approach for refractory AOSD.

In our study, we aim to describe, to our knowledge for the first time, the efficacy of tofacitinib in 14 …

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