Background/Purpose: Interstitial lung disease (ILD) is the leading cause of mortality and carries significant morbidity in connective tissue disease (CTD). Corticosteroids are often used as first line therapy, yet the use of additional immunosuppressive (IS) agents remains largely empiric. In this study we evaluated the effect of Rituximab (RTX) as rescue therapy on lung function in a cohort of patients with severe, progressive CTD-ILD.

Methods: We identified patients with CTD-ILD in the University of Chicago ILD registry who received conventional IS therapy with or without RTX from 2008 to 2018. Results of forced vital capacity (FVC) stratified by IS therapy pre- and post-RTX were compared.

Results: A total of 196 patients (65% female) with a mean age of 57.5 years were included. Rheumatoid arthritis (24.5%), mixed CTD (17.9%), and scleroderma (16.3%) were the most common CTD diagnosis. Of these, 96 received prednisone alone, 68 received azathioprine or mycophenolate without or without prednisone, and 32 received RTX in addition to these IS agents. In the RTX cohort, annualized FVC decline over 12 months pre-RTX was -6%, resulting in a mean FVC of 56% (95% CI=49%-62%) at the time of first RTX administration. One year post-RTX, there was a mean absolute increase of +5%, resulting in a post-RTX FVC of 61% (95% CI=49%-73%). In comparison, FVC remained stable to slightly improved in patients who did not receive RTX (0% in prednisone group; +2% in azathioprine/mycophenolate group) over this 12-month time period (Figure 1).

Conclusion: RTX may be an effective rescue therapy in patients with severe, progressive CTD-ILD refractory to conventional IS therapy.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/rituximab-as-rescue-therapy-in-treatment-refractory-ctd-ild/