Severe interstitial lung disease in connective tissue disease: rituximab as rescue therapy

GJ Keir, TM Maher, DM Hansell… - European …, 2012 - Eur Respiratory Soc
GJ Keir, TM Maher, DM Hansell, CP Denton, VH Ong, S Singh, AU Wells, EA Renzoni
European Respiratory Journal, 2012Eur Respiratory Soc
In very severe interstitial lung disease associated with connective tissue disease (CTD-ILD),
progressing despite maximal conventional immunosuppression, there is no effective
medical rescue therapy. The aim of the present study was to test whether rituximab, a
monoclonal antibody that depletes peripheral B lymphocytes, is effective as rescue therapy
in very severe CTD-ILD, unresponsive to conventional immunosuppression. We performed a
retrospective assessment of eight patients with severe and progressive CTD-ILD treated with …
In very severe interstitial lung disease associated with connective tissue disease (CTD-ILD), progressing despite maximal conventional immunosuppression, there is no effective medical rescue therapy.
The aim of the present study was to test whether rituximab, a monoclonal antibody that depletes peripheral B lymphocytes, is effective as rescue therapy in very severe CTD-ILD, unresponsive to conventional immunosuppression.
We performed a retrospective assessment of eight patients with severe and progressive CTD-ILD treated with rituximab. In six patients, change in pulmonary function tests (PFTs) compared with pre-rituximab levels, was assessed at 9–12 months post-treatment. In two patients, who were mechanically ventilated at the time of treatment, clinical and HRCT changes were assessed.
Seven out of eight patients had a favourable treatment response to rituximab, while in one patient disease severity did not change. In contrast with previous progression, we observed a median significant improvement of 22% in diffusing capacity for carbon monoxide (from a median baseline of 25%; range 16–32%; p=0.04), and a median significant improvement of 18% in forced vital capacity (from a median baseline of 45%; range 37–59%; p=0.03), in the 9–12 months following treatment with rituximab.
In very severe CTD-ILD unresponsive to conventional immunosuppression, rituximab may represent an effective, potentially life-saving, therapeutic intervention.
European Respiratory Society