SAT0468 Scleroderma -interstitial lung disease: does maintenance therapy make a difference
Authors: S.K. Sharma, A. Chattopadhyay, S. Naidu, V. Dhir, S. Jain
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Interstitial lung disease (ILD) is the commonest cause of death among Scleroderma (SSc) patients. The evidence regarding efficacy of cyclophosphamide(CYC) in SSc-ILD results mainly from two high-quality RCTs.<sup>1,2</sup> In the scleroderma lung study 1, after 12 months of therapy with oral CYC, a significant difference in mean forced vital capacity (FVC) was noted favouring CYC group. However, the effect waned off by the end of 24 months of follow up, indicating that maintenance therapy might help in preserving the initial benefits.<sup>3</sup> <h3>Objectives</h3> To study the long-term effect of maintenance therapy in SSc related interstitial lung disease (SSc-ILD) <h3>Methods</h3> This is a retrospective data analysis of forty-three patients with SSc-ILD divided into two groups, those who received maintenance therapy with either mycophenolate (MMF) or azathioprine(AZA) and the other group without any maintenance therapy following CYC induction. Response to treatment was classified as improved (≥10% increase in FVC from baseline), stable (change in FVC of 0%–10% from baseline) and worsened (≥10% decrease in FVC or fall of FVC to &lt;40% of predicted). Results were expressed as percentages for categorical variables and mean with standard deviation (SD) for continuous variables. Chi square test was used for comparison of responses between two groups of patients. Following induction patients were divided into groups: those They were assessed at three years. <h3>Results</h3> Baseline characteristics of the study population are shown in table 1. Maintenance therapy was given in 29 (67.4%) patients and among them favourable outcome (improve +stable) was noted in 23 (79.3%) patients and unfavourable outcome was noted in 6 (20.7%) patients. Fourteen (32.6%) patients did not receive maintenance therapy and among them, favourable outcome was noted in 4 (28.6%) patients and unfavourable outcome in 10 (71.4%) patients. This difference in the rates of favourable outcome between both the groups was statistically significant (p=0.001). Figure 1 summarises the response rates of the patients of these two groups. <h3>Conclusions</h3> Maintenance therapy up to 3 years (either with AZA or MMF) following IV-CYC induction, resulted in a favourable outcome and helps in preserving the initial beneficial effects achieved with IV CYC. <h3>References</h3> [1] Tashkin DP, Elashoff R, Clements PJ, et al. Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med2007;176:1026–34. [2] Hoyles RK, Ellis RW, Wellsbury J, et al. A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum2006;54:3962–70. [3] Tashkin DP, Elashoff R, Clements PJ, et al. Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med2007;176:1026–34. <h3>Disclosure of Interest</h3> None declared