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Updated: May 5, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Rituximab in severe, treatment-refractory interstitial lung disease
Gregory J Keir1, Toby M Maher, Damien Ming
1Royal Brompton Hospital, London, UK; Princess Alexandra Hospital, Brisbane, Queensland, Australia.
Rituximab may be an effective rescue therapy for severe, progressive interstitial lung disease (ILD) unresponsive to standard treatments. This study observed improved lung function and stability after rituximab administration in non-idiopathic pulmonary fibrosis ILD patients.
Area of Science:
- Pulmonology
- Immunology
- Rheumatology
Background:
- Severe interstitial lung disease (ILD) poses a significant challenge, especially when progressing despite conventional immunosuppression.
- Rituximab, a B-lymphocyte depleting monoclonal antibody, is explored as a potential rescue therapy for such refractory cases.
- Idiopathic pulmonary fibrosis (IPF) is excluded, focusing on other ILD etiologies.
Purpose of the Study:
- To assess the efficacy of rituximab as a rescue therapy in patients with severe, progressive ILD.
- To evaluate changes in pulmonary function following rituximab treatment.
- To analyze the safety profile of rituximab in this patient cohort.
Main Methods:
- Retrospective analysis of 50 patients with severe, progressive ILD (excluding IPF) treated with rituximab from 2010-2012.
- Comparison of pulmonary function tests (forced vital capacity and diffusing capacity for carbon monoxide) before and 6-12 months after rituximab treatment.
- Assessment of ILD aetiologies, including connective tissue disease, hypersensitivity pneumonitis, and miscellaneous conditions.
Main Results:
- Patients presented with severe physiological impairment (median FVC 44.0%, DLCO 24.5%).
- Following rituximab, a median improvement in FVC of 6.7% (P < 0.01) and stability in DLCO (0% change, P < 0.01) were observed at 6-12 months, contrasting with prior decline.
- Two patients experienced serious infections; 10 patients died due to ILD progression.
Conclusions:
- Rituximab demonstrates potential as an effective therapeutic intervention for severe, progressive non-IPF ILD unresponsive to conventional immunosuppression.
- Further prospective, controlled trials are necessary to confirm these findings and evaluate safety outcomes.
- Rituximab may represent a valuable option for patients with limited treatment alternatives.
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