Introduction Severe interstitial lung disease associated with systemic sclerosis (SSc-ILD) often has an inexorably progressive course. Prevention or retardation of disease progression (as seen in both SLS and the FAST trials) is the only realistic treatment goal in most cases. Rituximab is a B- lymphocyte depleting monoclonal antibody which has proven efficacy in a spectrum of treatment-refractory interstitial lung diseases. Data on the impact of rituximab therapy on SSc-ILD outcomes are limited.
Methods 18 patients with severe progressive SSc-ILD, despite conventional immunosuppression, were studied retrospectively. Serial change in FVC and DLco was quantified as percentage relative change from baseline, Pulmonary function trends pre (3–17 months) and post (3–11 months) Rituximab therapy were compared using paired t-testing.
Results 18 patients (four male), with a median age 57.5 (±15.9) received treatment with rituximab between 2012 and 2014. The median follow-up period was 7.96 (range 3.1–11.2) months. One patient died from heart failure. Rituximab was well tolerated. At the time of rituximab treatment, patients had severe pulmonary function impairment (median FVC 50.5%, range 36–84%; median DLco 25%, range 14–41%). On paired testing, there was a reduction in serial FVC decline following Rituximab therapy (-10.1% ± 7.8% versus -1.5% ± 8.7%, p = 0.01) and a similar reduction in serial DLco decline (-15.6% ± 16.8% vs. 1.0% ± 27.2%, p = 0.05).
Conclusion The addition of Rituximab was associated with a significant reduction in serial pulmonary function decline in patients with advanced progressive SSc-ILD, not controlled by intense conventional immunomodulation. These findings provide further support for the use of Rituximab as rescue therapy in severe SSc-ILD.
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