LAUSR

Background: The American Thoracic Society convened an international, multidisciplinary panel to develop clinical practice guidelines for the treatment of systemic sclerosis-associated interstitial lung disease (SSc-ILD). Objective: To conduct a systematic review and evaluate the literature to determine whether patients with SSc-ILD should be treated with mycophenolate. Data Sources: A literature search was conducted across MEDLINE, EMBASE, and CENTRAL databases through June 2022 for studies using mycophenolate to treat patients with SSc-ILD. Data Extraction: Mortality, disease progression, quality of life, and adverse event data were extracted, and meta-analyses performed when possible. The Grading of Recommendations, Assessment, Development and Evaluation (GRADE) Working Group method was used to assess the quality of evidence. Synthesis: The literature review resulted in 7 studies fitting the inclusion criteria. The systematic review and meta-analyses revealed changes in forced vital capacity (FVC) % predicted (mean difference [MD] 5.4%; 95% confidence interval [CI] 3.3%, 7.5%), diffusing capacity of the lung for carbon monoxide (DLCO) % predicted (MD 4.64%; 95% CI 0.54%, 8.74%), and breathlessness score (MD 1.99; 95% CI 0.36, 3.62) favored mycophenolate over placebo. Risk of anemia (Relative Risk [RR] 2.3; 95% CI 1.2, 71.4) was higher with mycophenolate. There were no significant differences between mycophenolate and cyclophosphamide except risk of premature discontinuation (RR 0.6; 95% CI 0.4, 0.9) and leukopenia (RR 0.1; 95% CI 0.05, 0.4) favored mycophenolate. The quality of evidence was moderate to very low GRADE. Conclusions: Mycophenolate use in patients with SSc-ILD is associated with statistically significant improvements in disease progression and quality-of-life measures compared to placebo. There were no differences in mortality, disease progression, or quality-of-life compared to cyclophosphamide, but there were fewer adverse events. The quality of evidence is very low.