Background Rituximab (RTX) is a chimeric mAb against human CD20 that depletes peripheral B cells and introduced for systemic rheumatic diseases. Basic research and clinical studies have provided preliminary data pointing that RTX might be a therapeutic option for patients with systemic sclerosis. However there are no enough evidences for systemic sclerosis-associated interstitial lung disease (SSc-ILD). No formal randomized studies are available to indicate what the optimal strategy is for patients with SSc-ILD
Objectives We evaluate the effectiveness and safety profiles RTX for SSc
Methods Prospective study of thirty consecutive patients with SSc-ILD was performed. All patients had severe ILD as indicated by findings in chest HRCT and pulmonary functional tests. There were two indications for RTX: early progressive diffuse SS (10 patients) and ILD refractory to usual treatment (20 patients). Of the 30 ptatients received RTX two patients died and one have lost for follow up. The total 27 ptatients (26 female) were included and followed for 12 months. Fourteen patients received 2g RTX, 11 patients – 1 g and 2 patients - 0, 5 g. Demographic, clinical and laboratory data was obtained
Results Baseline characteristics: mean age was 45, 7±13, disease duration of 7, 6±4, 7 (1, 5-18, 5) years. Patients had diffuse (13) and limited (14) SSc, 17 had positive Scl-70- antibodies. Six patients had pulmonary hypertension (sPAP increase by echo >45 mmHg). At the 12 months evaluation time point a significant improvement of FVC (%) was found: 70±17, 8 vs 77±19, 8 at baseline vs 12 months respectively, p=0,001. There were no differences in concern to the values of FVC (% predicted) before and at 12 months between diffuse (71,5±15 vs 79±17, p=0,036 and limited (69±20 vs 75±22, p=0,01) forms of SSc. Patients with long-term duration of the disease (more than 8 years) had lowest FVC at baseline and the improvement in a 12 months was not statistically significant (FVC 63,5±14 vs 67,4 ±14 before and at 12 months respectively, p=0,11). The highest increasing of FVC after treatment had patients with disease duration ≤ 5 year, differences were 11%. The improvement seen in DCLO (% predicted) did not reach statistical significance: 38, 3±14 at baseline vs 41, 6±12 at 12 months, p=0,058. Skins thickening (mMRSM), 6MWT, level of B-cells and Scl-70 antibodies did not change significantly. The activity index significantly decreased (p=0,006). Treatment was well tolerated. Two cases of mild infusion reactions, three cases of respiratory tract infection, one case of herpes zoster and two cases of depression of ST by ECG were recorded.
Conclusions RTX could be effective and relatively safe in patients with diffuse and limited SSc-ILD. Our preliminary data suggests that effect of RTX on lung function could not be realized in patients with prolonged ILD and severe impairment of lung function at baseline. We suspect that absence of beneficial effect in concerrn to DCLO and other parameters of the disease in our group are related with incomplete cycles of RTX.
Disclosure of Interest None Declared