Rituximab in the treatment of progressive interstitial lung disease associated with the antisynthetase syndrome

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 tha...

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Detalles Bibliográficos
Autores: Narváez, Javier, Cañadillas, Elena, Castellví, Ivan, Alegre, Juan José, Vincens Zygmunt, Vanesa, Bermudo, Guadalupe, Vidal Montal, Paola, Molina Molina, María, Nolla Solé, Joan Miquel
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2024
País:España
Institución:Universidad de Barcelona
Repositorio:Dipòsit Digital de la UB
OAI Identifier:oai:diposit.ub.edu:2445/214883
Acceso en línea:https://hdl.handle.net/2445/214883
Access Level:acceso abierto
Palabra clave:Malalties del pulmó
Miositis
Pulmonary diseases
Myositis
Descripción
Sumario: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: triangle%pFVC +6.29% (95% CI: -10.07 to 2.51; p=0.002 compared to T0) and triangle%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: triangle%pFVC: +9.93% (95% CI: -15.61 to -4.25; p=0.002) and triangle%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. 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: triangle%pFVC +6.29% (95% CI: -10.07 to 2.51; p=0.002 compared to T0) and triangle%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: triangle%pFVC: +9.93% (95% CI: -15.61 to -4.25; p=0.002) and triangle%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. Conclusion 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.