AB0604 Rituximab experience in patients with longstanding systemic sclerosis-associated interstitial lung disease: a series of 14 patients

Abstract

BackgroundInterstitial 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).ObjectivesObjective 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.MethodsWe 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.ResultsMedian (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].Table 1.Demographic, clinical and laboratory data of patientsAge/SexDisease duration, yearsCutaneous subsetAutoantibodiesPrevious immunosupresive treatmentRTX cyclesFollow-up after RTX, monthsFVC (predicted%) Before RTXAfter RTX 52/F7.0DiffuseANA, Scl-70CYC, MMF212444439/M10.1DiffuseANA, Scl-70MMF424757955/F5.0DiffuseANA, Scl-70MMF424757043/F16.6LimitedANA, Scl-70CYC, MMF16384750/F4.6LimitedANA, Scl-70CYC, MMF424525765/F13,0DiffuseANA, Scl-70CYC, MMF16424148/F5.7LimitedANA, Scl-70CYC424676454/F18.9DiffuseANA, Scl-70CYC, MMF212395753/F15.0LimitedANA, Scl-70CYC, MMF530534456/F5,1LimitedANA, Scl-70CYC16404552/F8.2LimitedANA–312595918/F11.2DiffuseANA, Scl-70MMF16637362/F4.6LimitedANA, Scl-70CYC, MMF318516154/F13.1LimitedANA, Scl-70CYC, MMF5305467FVC, forced vital capacity; ANA, antinuclear antibody; Scl-70, antitopoisomerase-1 antibody; CYC, cyclophosphamide; MMF,mycophenolate mofetil; RTX, rituximab.ConclusionsIn 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 InterestNone declared

Effect Size of Rituximab on Pulmonary Function in the Treatment of Connective-Tissue Disease-Related Interstitial Lung Disease: A Systematic Review and Meta-Analysis

“…Incidentally, studies involving SSc-ILD patients were also the largest represented subtype in our meta-analysis. FVC% appeared to improve after RTX therapy in the combined meta-analysis 13,15,21,22,30 inclusive of the only RCT in our systematic review 19 . Divergent ndings though were reported in 23 patients of a subgroup in the study by Lepri et al 15 .…”

“…A total of 3806 individual citations were found and screened, resulting in the inclusion of 20 studies for systematic review, 14 of which were included speci cally in the quantitative meta-analysis of treatment effect size on lung function [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32] . Study selection is presented in Fig.…”

Safety and efficacy of Rituximab in systemic sclerosis: A systematic review and meta-analysis

“…Lots of clinical studies have been conducted to comprehensively evaluate the efficacy and safety of this therapeutic strategy in multiple involved tissues and organs. As for Rituximab in SSc, current primary outcome often assesses the cutaneous and pulmonary function due to their importance for clinical mortality 13 . Indisputably, the articular, cardiovascular, renal, and gastrointestinal organ involvements are of great effect on patients' clinical outcome 14 .…”

Safety and Efficacy of Rituximab in Systemic Sclerosis: A Systematic Review and Meta-analysis

“…The safety profile of Rituximab was satisfactory in our SSc patients, only a few severe adverse events were related to Rituximab throughout the treatment. to their importance for clinical mortality 13 . Indisputably, the articular, cardiovascular, renal, and gastrointestinal organ involvements are of great effect on patients' clinical outcome 14 .…”