TY - JOUR T1 - Tofacitinib in refractory adult-onset Still’s disease: 14 cases from a single centre in China JF - Annals of the Rheumatic Diseases JO - Ann Rheum Dis SP - 842 LP - 844 DO - 10.1136/annrheumdis-2019-216699 VL - 79 IS - 6 AU - Qiongyi Hu AU - Mengyan Wang AU - Jinchao Jia AU - Jialin Teng AU - Huihui Chi AU - Tingting Liu AU - Hong-lei Liu AU - Xiaobing Cheng AU - Junna Ye AU - Yutong Su AU - Yue Sun AU - Zhuochao Zhou AU - Liyan Wan AU - Zhihong Wang AU - Fan Wang AU - Hui Shi AU - Chengde Yang Y1 - 2020/06/01 UR - http://ard.bmj.com/content/79/6/842.abstract N2 - 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 … ER -