TY - JOUR T1 - A Retrospective Analysis of Outcome in MDA5-Related Interstitial Lung Disease Treated with Tofacitinib or Tacrolimus JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.220367 SP - jrheum.220367 AU - Li Fan AU - Wenting Lyu AU - Huarui Liu AU - Hanyi Jiang AU - Lulu Chen AU - Yin Liu AU - Yi Zhuang AU - Mei Huang AU - Min Cao AU - Hourong Cai AU - Yonglong Xiao AU - Jinghong Dai Y1 - 2022/08/15 UR - http://www.jrheum.org/content/early/2022/08/09/jrheum.220367.abstract N2 - Objective The efficacy of tofacitinib in early diagnosis of MDA5-ILD has been described. But whether tofacitinib exposure is associated with a reduced one-year mortality remains undetermined. Methods Patients diagnosed as MDA5-ILD receiving tofacitinib or tacrolimus treatment were included. Cox proportional hazards model adjusted for age, sex, smoking history, anti-MDA5 antibody titers, concurrent use of other steroids sparing agents was performed to compare all-cause mortality and to investigate the risk factors predicting 1-year mortality in the two treatment groups. Results During the study period, twenty-six patients were treated with tofacitinib and thirty-five with tacrolimus. The 6-month and 1-year mortality in tofacitinib group were significantly lower than those in tacrolimus group (38.5% vs. 62.9%, p=0.028; 44.0% vs. 65.7%, p=0.031, respectively). There were thirteen patients diagnosed as rapidly progressive-ILD (RPILD) in tofacitinib group and twenty-two in tacrolimus group. The majority of death occurred in patients with RPILD. The 6-month and 1-year mortality of patients with RPILD in tofacitinib group were also lower than those in tacrolimus group (76.9% and 95.5%, p=0.021; 84.6% and 100.0%, p=0.017). The adjusted model showed tofacitinib exposure was associated with a lower risk for 1-year mortality (HR0.438, 95% CI 0.200-0.960, p=0.039). While the incidence of adverse events and medication discontinuation rates between the two groups were similar (73.1% and 74.3%, p=1.000; 23.1% and 14.3%, p=0.504). Conclusion Our observational study showed tofacitinib use might have a potential impact on improving the outcomes of MDA5-ILD. Future clinical trials are needed to assess the long-term efficacy and tolerability of tofacitinib. ER -