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. (23rd January 2014) - Balbir-Gurman, A.
Abstract : 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<0.05. Results: A total of 225 SSc pts were registered in the EUSTAR database at our site, 41pts were had an active ILD, data on 28 pts started CYC before year 2007 (17/28- before year 2004) were eligible. Age, disease duration, and follow-up period were (mean +/- SD) 50.7 +/- 12.7 years (yrs), 16.3 +/- 17.9 months, 6.5 +/- 6 yrs respectively. Eight pts died (6 pts due to SSc). Mean cumulative CYC dose was 8.96 +/- 3.8 G; in 12 pts the treatment was followed by another non-biological DMARD. Three pts developed adverse events: 2-pneumonia, 1-hepatitis B reactivation and ankle Kaposi sarcoma, 1-premature menopause. Main changes in FVC, DLCO, PAP and mRSS were observed in first 4 yrs after CYC treatment with mild additional reduction in the next three yrs. Annual changes rate in FVC, DLCO, mRSS, and PAP are presented in Table 1 . Among presenting features only elevated CK correlated with FVC, DLCO and PAP (p=0.04, p=0.026 and p=0.028 respectively). Reduction in mRSS was significant (p=0.023) at any cumulative CYC dose, but not changes in FVC, DLCO and PAP. Conclusions: Course of CYC IV infusions did not prevent FVC and DLCO reduction as well as PASP elevation in the next 4 yrs of follow-up. CYC rapidly and significantly improved mRSS. Cumulative doses of CYC above 6G had not additional influence on FVC and DLCO. In SSc-ILD follow-up longer than 4 years indicates a need for maintenance therapy after stabilization of pulmonary function tests with CYC. Disclosure of Interest : None Declared