Abstract

BackgroundNowadays, there is already published a lot of data on the treatment of systemic sclerosis (SSc) with rituximab (RTX). Recent studies reported about improvement of skin fibrosis and interstitial lung disease associated with SSc (ILD-SSc) on RTX treatment. However, there is not enough data on changes of pulmonary artery systolic pressure (PASP) in SSc patients (pts) on RTX therapy.ObjectivesTo evaluate changes of PASP in ILD-SSc pts on RTX therapy.Methods30 pts with initially elevated PASP (detected as ≥35mmHg by echocardiography) were retrospectively selected from a group of 103 SSc pts who received RTX as a therapy for ILD. The mean follow-up period was 25.3±2.4 months. The mean age was 52.6 years (30-65), female-26 pts (87%), the diffuse cutaneous subset of the disease had 13 pts (43%). The mean disease duration was 7.2±6.3 years. The cumulative mean dose of RTX was 2.3±1.1 grams. All pts received prednisolone at a dose of 11.3±3.7mg/day, immunosuppressants received 47% of them. All pts were positive for ANA. An assessment of basic parameters was obtained at baseline (Point 0), after 13.6±2.7 months (Point 1) and at the end of follow-up (25.3±2.4 months - Point 2). The results at baseline and at the end of the follow-up are presented in the form of mean values.ResultsThere was already an improvement of all main parameters at the point 1 and further - by the end of follow-up (Table 1). There was a significant decrease in PASP, which was accompanied by the improvement of lung function - increase in forced vital capacity % predicted (FVC), stabilization of diffusion capacity for carbon monoxide % predicted (DLCO). A moderate negative statistically significant correlation was found between the PASP and FVC (r=-0,522; p=0,003). There was a significant increase in 6-minute walk distance (6-mwd), ejection fraction of left ventricular (ER), activity index (EScSG-AI) and a decrease of Rodnan skin score.Table 1.Changes of the main parameters on RTX treatment.ParametersPoint 0 (baseline)Point 1 (13.6±2.7 month)Point 2 (25.6±2.4 month)P Point 0-1P Point 0-2FVC, %, M±σ75.6±20.182.5±19.288.2±22.50.010.002PASP, mmHg, M±σ48.2±13.844.4±12.339.5±11.80.0080.004DLCO, %, M±σ42.7±16.242.6±15.244.9±15.6NSNSER, %, M±σ61.6±5.563.5±6.265.8±5.70.020.01Rodnan skin score, M±σ11.1±12.47.2±8.16.1±7.60.0030.00016-mwd, m, M±σ419.6±103453.8±85458.8±78.40.020.02Activity index (EScSG-AI), M±σ3.3±1.51.5±1.31.4±1.10.0010.001Cumulative mean dose of RTX, g, M±σ-1.65±0.692.3±1.1--ConclusionIn our study, the PASP significantly decreased already in a year after initiation of RTX therapy and further there was an improvement of this parameter at the end of the observation. Decrease of PASP correlated with an increase of FVC and was accompanied by an increase of 6-minute walk distance, ejection fraction of left ventricular, improvement of Rodnan skin score and disease activity index. RTX could be considered as a potentially effective in combined treatment of ILD-SSc, complicated with pulmonary hypertension.Disclosure of InterestsNone declared

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