Mycophenolate mofetil for scleroderma-related interstitial lung disease: A real world experience

Background and objective: Interstitial lung disease (ILD) remains the number one cause of mortality in scleroderma (SSc). Our goal was to determine the effectiveness of mycophenolate mofetil (MMF) in treating SSc-ILD in a retrospective study. Methods: A retrospective, computer-assisted search was performed to identify patients with SSc-ILD treated with MMF from 1997 through 2014. We used a novel software tool, Computer-Aided Lung Informatics for Pathology Evaluation and Rating (CALIPER), to quantify parenchymal lung abnormalities on high-resolution computed tomography. Lung function was evaluated at baseline, 6, 12, and 24 months of MMF therapy. Results: We identified 46 patients (28 females) with SSc-ILD (mean age at diagnosis 55 y) treated with MMF for at least 1 year (majority on 2 gm/day). Twenty-one patients (45.7%) stopped using MMF during the follow up period after the first 12 months, and they took MMF for a median of 2.12 years (range, 0.91–8.93 years). Only 4 discontinued MMF because of disease progression. Compared to baseline, the mean percentage change in forced vital capacity (95% CI) at 6, 12, and 24 months, respectively, was 1.01% (−2.38%-4.39%) (n = 26), 2.06% (−1.09%-5.22%) (n = 31), and −0.07% (−3.31%-3.17%) (n = 30), and the mean percentage change in ILD as measured by CALIPER (95% CI) was −5.40% (−18.62%-7.83%) (n = 18), −1.51% (−14.69%-11.68%) (n = 17), and −8.35% (−20.71%-4.02%) (n = 22).The mean right ventricular systolic pressure (RVSP) remained stable over the study period. Conclusions: MMF is well tolerated and slows the rate of decline in lung function in SSc-ILD patients, even at doses lower at 3 g/day.