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

• Published in: PloS one Vol. 12; no. 5; p. e0177107
• Main Authors: Baqir, Misbah, Makol, Ashima, Osborn, Thomas G, Bartholmai, Brian J, Ryu, Jay H
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 25.05.2017; Public Library of Science (PLoS)
• Subjects: Accessibility; Adult; Aged; Alveoli; Alveolitis; Antibiotics, Antineoplastic - therapeutic use; Arthritis; Assessments; Azathioprine; Biology and Life Sciences; Blood pressure; Bone marrow; Bronchus; Care and treatment; Chest; Clinical trials; Comfort; Complications and side effects; Computed tomography; Computer programs; Connective tissues; Critical care; Development and progression; Diagnosis; Dosage and administration; Drug dosages; Drug therapy; Dyspnea; Echocardiography; Effectiveness; Evaluation; Female; Glucocorticoids; Health aspects; Heart; Hemorrhage; High resolution; Humans; Hypertension; Incidence; Inflammation; Internal medicine; Interstitial lung diseases; Kidneys; Lung - drug effects; Lung diseases; Lung Diseases, Interstitial - drug therapy; Male; Medical records; Medicine; Medicine and Health Sciences; Middle Aged; Mycophenolate mofetil; Mycophenolic Acid - therapeutic use; Pneumonia; Pulmonary fibrosis; Pulmonary hypertension; Quality of life; Radiology; Rats; Research and Analysis Methods; Respiration; Respiratory function; Retrospective Studies; Rheumatology; Risk factors; Safety; Scleroderma; Scleroderma (Disease); Scleroderma, Systemic - drug therapy; Sexually transmitted diseases; Side effects; Skin; Sodium; Software development tools; STD; Studies; Survival; Teratogenicity; Toxicity; Ventricle; Vital Capacity - drug effects; Young Adult; United States; United States > US; Minnesota
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0177107

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.