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Observational Study
. 2024 Jun 18;26(1):122.
doi: 10.1186/s13075-024-03353-2.

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

Javier Narváez  1 , Elena Cañadillas  2 , Iván Castellví  3 , Juan José Alegre  4 , Vanesa Vicens-Zygmunt  5 , Guadalupe Bermudo  5 , Paola Vidal-Montal  6 , María Molina Molina  5 , Joan Miquel Nolla  6
Affiliations
Observational Study

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

Javier Narváez et al. Arthritis Res Ther. .

Erratum in

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.

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.

Keywords: Antisynthetase syndrome; Progressive interstitial lung disease; Treatment; Rituximab.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Evolution of the predicted forced vital capacity (%pFVC) and the predicted diffusing capacity for carbon monoxide corrected for haemoglobin (%pDLCO) before initiation of RTX and after 1 year of treatment
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