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). 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. 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. 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.

中文翻译：

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利妥昔单抗治疗与抗合成酶综合征相关的进行性间质性肺疾病


评估利妥昔单抗 (RTX) 作为抗合成酶综合征和进行性间质性肺病 (ASS-ILD) 患者救援疗法的真实长期有效性。对一组 ASS-ILD 患者进行的多中心观察性回顾性纵向研究，这些患者尽管接受了糖皮质激素和免疫抑制剂治疗，但由于 ILD 复发或持续进展而开始接受 RTX 治疗。对二十八名患者进行了分析。检查整个研究人群，在 RTX 治疗前，从 ASS-ILD 诊断到开始 RTX 治疗 (T0) 的 %pFVC 和 %pDLCO 平均下降分别为 -6.44% 和 -14.85%。治疗六个月后，RTX 逆转了肺功能测试 (PFT) 参数的下降：Δ%pFVC +6.29%（95% CI：-10.07 至 2.51；与 T0 相比，p=0.002）和 Δ%pDLCO +6.15%（ 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 缓解：Δ%pFVC：+9.93%（95% CI：-15.61 至 -4.25；p=0.002）和 Δ%pDLCO：+7.66% （95% CI：-11.67 至 -3.65；p<0.001）。此外，泼尼松的中位剂量也显着减少，18%的病例可以暂停使用。 33% 在治疗开始时需要氧疗的患者可以停止氧疗。不良事件发生率达到28.5%。根据我们的结果，对于大多数对常规治疗无反应的复发性或进展性 ASS-ILD 患者，RTX 作为挽救疗法似乎是有效的。大多数患者对 RTX 的耐受性良好。