Mycophenolate mofetil for scleroderma-related interstitial lung disease: A real world experienceReport as inadecuate

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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.


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.


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.


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.

Author: Misbah Baqir , Ashima Makol, Thomas G. Osborn, Brian J. Bartholmai, Jay H. Ryu



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