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Abstract：
OBJECTIVE: To assess the real-world, long-term effectiveness of rituximab (RTX) as a rescue therapy in patients with antisynthetase syndrome and progressive interstitial lung disease (ASS-ILD).
METHODS: Multicentre observational retrospective longitudinal study of a cohort of patients with ASS-ILD that started treatment with RTX due to recurrent or ongoing progressive ILD despite therapy with glucocorticoids and immunosuppressants.
RESULTS: Twenty-eight patients were analyzed. Examining the entire study population, before treatment with RTX the mean decline in %pFVC and %pDLCO from the ASS-ILD diagnosis to the initiation of RTX treatment (T0) was -6.44% and -14.85%, respectively. After six months of treatment, RTX reversed the decline in pulmonary function test (PFT) parameters: ∆%pFVC +6.29% (95% CI: -10.07 to 2.51; p=0.002 compared to T0) and ∆%pDLCO +6.15% (95% CI: -10.86 to -1.43; p=0.013). Twenty-four patients completed one year of therapy and 22 two years, maintaining the response in PFT: ∆%pFVC: +9.93% (95% CI: -15.61 to -4.25; p=0.002) and ∆%pDLCO: +7.66% (95% CI: -11.67 to -3.65; p<0.001). In addition, there was a significant reduction in the median dose of prednisone, and it could be suspended in 18% of cases. In 33% of patients who required oxygen therapy at the start of treatment, it could be discontinued. The frequency of adverse events reached 28.5% of cases.
CONCLUSIONS: Based on our results, RTX appears to be effective as rescue therapy in most patients with recurrent or progressive ASS-ILD unresponsive to conventional treatment. The use of RTX was well tolerated in the majority of patients.
METHODS: Multicentre observational retrospective longitudinal study of a cohort of patients with ASS-ILD that started treatment with RTX due to recurrent or ongoing progressive ILD despite therapy with glucocorticoids and immunosuppressants.
RESULTS: Twenty-eight patients were analyzed. Examining the entire study population, before treatment with RTX the mean decline in %pFVC and %pDLCO from the ASS-ILD diagnosis to the initiation of RTX treatment (T0) was -6.44% and -14.85%, respectively. After six months of treatment, RTX reversed the decline in pulmonary function test (PFT) parameters: ∆%pFVC +6.29% (95% CI: -10.07 to 2.51; p=0.002 compared to T0) and ∆%pDLCO +6.15% (95% CI: -10.86 to -1.43; p=0.013). Twenty-four patients completed one year of therapy and 22 two years, maintaining the response in PFT: ∆%pFVC: +9.93% (95% CI: -15.61 to -4.25; p=0.002) and ∆%pDLCO: +7.66% (95% CI: -11.67 to -3.65; p<0.001). In addition, there was a significant reduction in the median dose of prednisone, and it could be suspended in 18% of cases. In 33% of patients who required oxygen therapy at the start of treatment, it could be discontinued. The frequency of adverse events reached 28.5% of cases.
CONCLUSIONS: Based on our results, RTX appears to be effective as rescue therapy in most patients with recurrent or progressive ASS-ILD unresponsive to conventional treatment. The use of RTX was well tolerated in the majority of patients.
摘要：
目的：为了评估现实世界，利妥昔单抗（RTX）作为抗合成酶综合征和进行性间质性肺病（ASS-ILD）患者的抢救治疗的长期有效性。
方法：对一组ASS-ILD患者进行多中心观察性回顾性纵向研究，尽管使用了糖皮质激素和免疫抑制剂，但由于复发性或持续进行性ILD而开始使用RTX治疗。
结果：对28例患者进行分析。检查整个研究人群，在使用RTX治疗之前，从ASS-ILD诊断到开始RTX治疗（T0）的％pFVC和％pDLCO的平均下降分别为-6.44％和-14.85％，分别。经过6个月的治疗,RTX逆转了肺功能测试（PFT）参数的下降：Δ％pFVC6.29％（95％CI：-10.07至2.51；与T0相比，p=0.002）和Δ％pDLCO6.15％（95％CI：-10.86至-1.43；p=0.013）。24名患者完成了一年的治疗，22名患者完成了两年的治疗，维持PFT中的反应：△%pFVC：+9.93%（95%CI：-15.61至-4.25；p=0.002）和△%pDLCO：+7.66%（95%CI：-11.67至-3.65；p<0.001）。此外,泼尼松的中位剂量显着减少，在18%的案件中，它可能会被暂停。在治疗开始时需要氧气治疗的患者中，有33％它可以停止。不良事件的发生频率达到28.5％的病例。
结论：根据我们的结果，RTX似乎在大多数对常规治疗无反应的复发性或进行性ASS-ILD患者中作为抢救治疗有效。大多数患者对RTX的使用具有良好的耐受性。
方法：对一组ASS-ILD患者进行多中心观察性回顾性纵向研究，尽管使用了糖皮质激素和免疫抑制剂，但由于复发性或持续进行性ILD而开始使用RTX治疗。
结果：对28例患者进行分析。检查整个研究人群，在使用RTX治疗之前，从ASS-ILD诊断到开始RTX治疗（T0）的％pFVC和％pDLCO的平均下降分别为-6.44％和-14.85％，分别。经过6个月的治疗,RTX逆转了肺功能测试（PFT）参数的下降：Δ％pFVC6.29％（95％CI：-10.07至2.51；与T0相比，p=0.002）和Δ％pDLCO6.15％（95％CI：-10.86至-1.43；p=0.013）。24名患者完成了一年的治疗，22名患者完成了两年的治疗，维持PFT中的反应：△%pFVC：+9.93%（95%CI：-15.61至-4.25；p=0.002）和△%pDLCO：+7.66%（95%CI：-11.67至-3.65；p<0.001）。此外,泼尼松的中位剂量显着减少，在18%的案件中，它可能会被暂停。在治疗开始时需要氧气治疗的患者中，有33％它可以停止。不良事件的发生频率达到28.5％的病例。
结论：根据我们的结果，RTX似乎在大多数对常规治疗无反应的复发性或进行性ASS-ILD患者中作为抢救治疗有效。大多数患者对RTX的使用具有良好的耐受性。
