Mycophenolate mofetil for sclerodermarelated interstitial lung disease: A real world experience

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Abstract

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.

Original languageEnglish (US)
Article numbere0177107
JournalPLoS One
Volume12
Issue number5
DOIs
StatePublished - May 1 2017

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Mycophenolic Acid
Pulmonary diseases
Interstitial Lung Diseases
respiratory tract diseases
Lung
lung function
lungs
Informatics
Pathology
computed tomography
retrospective studies
disease course
Vital Capacity
Ventricular Pressure
Tomography
Disease Progression
therapeutics
Software
Retrospective Studies
dosage

ASJC Scopus subject areas

  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • Agricultural and Biological Sciences(all)

Cite this

@article{7f953d282965425b854f7b4c4d7deec9,
title = "Mycophenolate mofetil for sclerodermarelated interstitial lung disease: A real world experience",
abstract = "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.",
author = "Misbah Baqir and Ashima Makol and Thomas Osborn and Bartholmai, {Brian Jack} and Ryu, {Jay H}",
year = "2017",
month = "5",
day = "1",
doi = "10.1371/journal.pone.0177107",
language = "English (US)",
volume = "12",
journal = "PLoS One",
issn = "1932-6203",
publisher = "Public Library of Science",
number = "5",

}

TY - JOUR

T1 - Mycophenolate mofetil for sclerodermarelated interstitial lung disease

T2 - A real world experience

AU - Baqir, Misbah

AU - Makol, Ashima

AU - Osborn, Thomas

AU - Bartholmai, Brian Jack

AU - Ryu, Jay H

PY - 2017/5/1

Y1 - 2017/5/1

N2 - 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.

AB - 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.

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