Effect of mycophenolate mofetil on pulmonary function in scleroderma-associated interstitial lung disease

Objective: We sought to determine the effectiveness of mycophenolate mofetil (MMF) in scleroderma-associated interstitial lung disease (SSc-ILD). Methods: We retrospectively identified patients who met criteria for systemic sclerosis, had evidence of SSc-ILD on chest CT, received > 1 g/d of MMF for ≥ 6 months, and had pulmonary (DLCO) at treatment onset were compared with VC and DLCO values 12 months before and 12 months after treatment onset. Twelve-month values were imputed from regression lines generated using all VC and DLCO measurements made in the 24-month period either prior to or following treatment onset. Results: Among 13 patients who met inclusion criteria, MMF was associated with a significant improvement in VC (mean, + 159 mL; confidence interval [CI], + 30 to + 289 mL; and + 4% of the predicted normal value; CI, + 2 to + 7%) after 12 months of treatment. In contrast, patients had a significant decrease in VC (mean, - 239 mL; CI, - 477 to - 0.5 mL; and - 5% of the predicted normal value; CI, - 11 to - 0.3%) in the 12 months prior to MMF treatment. DLCO did not change significantly during MMF treatment (mean, + 1% of the predicted normal value; CI, - 2 to + 5%) but decreased significantly in the 12 months prior to treatment (mean, - 5% of the predicted normal value; CI, - 10 to - 1%). Conclusion: These retrospective data suggest MMF improves VC in patients with SSc-ILD.