FRI0385 Long term follow-up after systemic sclerosis patients treated with intravenous cyclophosphamide pulse therapy for interstitial lung disease: a single eustar center (042) experience

Background Two RCT and several open-labeled studies demonstrated various efficacy of cyclophosphamide (CYC) for treatment of Systemic sclerosis-associated interstitial lung disease (SSc-ILD) with follow up for 1-4 years. Objectives To analyze the changes in pulmonary function tests, pulmonary artery pressure (PAP) estimated by Doppler echocardiography, and skin involvement measured by modified Rodnan’ skin score (mRSS) at 4-years, and 7-years follow-up after completion of at list 6 monthly treatments intravenous (IV) CYC of SSc-ILD. Methods The data on pts with active SSc-ILD (ground glass opacities and/or pulmonary fibrosis (PF) on chest HRCT and reduction in FVC and/or DLCO for more than 10% during 2 consecutive visits) who started IV CYC before year 2007 were analyzed. Student T-test, Mann-Whitney U-test and Wilcoxon Signed Ranks tests were used for statistical analysis with significance measured by p