Background Interstitial lung disease (ILD) is the leading cause of mortality in systemic sclerosis (SSc) patients. Treatment options are rather limited in SSc associated ILD (SSc-ILD).
Objectives Objective of this study was to report the experience of RTX treatment in a series of patients with longstanding SSc-ILD in whom unsatisfactory response in lung functions was noted under conventional treatments.
Methods We retrospectively reviewed charts of 197 SSc patients evaluated between April 2015 and November 2016. 14 patients who received rituximab (RTX) for SSc-ILD participated in this analysis. The severity of ILD based on PFTs was defined as follows; mild (FVC between 71% and 80% of predicted), moderate (FVC between 51% and 70% of predicted) and severe (FVC ≤50%). The extent of skin disease was clinically measured by using Modified Rodnan Skin Score (mRSS) tool. End of follow-up was considered as six months after the last RTX dose.
Results Median (IQR, interquartile range) age was 53.2 (46.8–55.5) and median disease duration was 9.1 (5.1–13.6) years. Median FVC was 52.5 (41.5–64.0) prior to RTX. At the end of follow-up, no significant change was revealed in FVC when compared with pre-RTX values [58.0 (44.7–58.7), p=0.065]. FVC was improved in four patients and stabilized remaining ten patients. All of the patients with improvement of PFTs had moderate or severe restrictive lung disease. High resolution thorax computed tomography (HRCT) findings remained stable in 7 and showed progression of ILD in 3 patients. In total, mRSS remained stable at the end of follow-up when compared with baseline [8.0 (5.2–12.2) vs. 6.0 (4.0–12.2),p=0,026].
Conclusions In this case series of SSc patients treated with RTX, improvement or stabilization of pulmonary functions was observed in most of SSc patients. RTX may be useful in SSc-ILD patients with longer disease duration and resistant to conventional immunsupresive therapies.
Disclosure of Interest None declared
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