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Abstract：
Idiopathic inflammatory myopathy (IIM)-associated interstitial lung disease (ILD) is often rapidly progressive with a poor prognosis; however, no standard therapeutic regimen has been identified. This study aimed to investigate the efficacy and safety of rituximab in IIM-ILD patients. Five patients who had been administered rituximab for IIM-ILD at least once between August 2016 and November 2021 were included. Lung function decline was compared one year before and after rituximab. Disease progression, defined as a greater than 10% relative decline in forced vital capacity (FVC) compared to the baseline, was also compared before and after treatment. Adverse events were recorded for safety analysis. Five IIM-ILD patients received eight cycles. FVC-predicted values significantly decreased from 6 months before rituximab administration to those at the baseline (54.1% predicted (pre-6 months) vs. 48.5% predicted (baseline), p = 0.043); however, the FVC decline stabilized after rituximab. The rate of disease progression before rituximab showed a tendency to decrease after rituximab (75% (before) vs. 12.5% (6 months after, p = 0.059) vs. 14.3% (12 months after, p = 0.102)). Three adverse events developed, but none resulted in death. Rituximab can stabilize lung function decline with tolerable safety in Korean IIM patients with refractory ILD.
摘要：
特发性炎性肌病（IIM）相关的间质性肺病（ILD）通常进展迅速，预后不良；然而，尚未确定标准治疗方案.本研究旨在探讨利妥昔单抗在IIM-ILD患者中的疗效和安全性。纳入了5名在2016年8月至2021年11月期间至少接受过一次利妥昔单抗治疗IIM-ILD的患者。比较利妥昔单抗前后一年的肺功能下降。疾病进展,定义为与基线相比，强迫肺活量（FVC）的相对下降超过10％，治疗前后也进行了比较。记录不良事件用于安全性分析。五名IIM-ILD患者接受了八个周期。FVC预测值从利妥昔单抗给药前6个月到基线时显着降低（预测的54.1％（6个月前）与48.5%预测(基线)，p=0.043)；然而，利妥昔单抗治疗后FVC下降趋于稳定。利妥昔单抗之前的疾病进展率在利妥昔单抗之后显示出降低的趋势（75％（之前）与12.5%(6个月后，p=0.059)与14.3%(12个月后，p=0.102））。发生了三个不良事件，但没有人导致死亡。利妥昔单抗可以稳定韩国IIM难治性ILD患者的肺功能下降，具有可耐受的安全性。
