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.