Mycophenolate mofetil following cyclophosphamide in worsening systemic sclerosis-associated interstitial lung disease

Abstract

Objectives Randomized controlled trials have shown that cyclophosphamide (CYC) was an option in systemic sclerosis-associated interstitial lung disease (SSc-ILD). The observed improvement disappeared after CYC was stopped suggesting that a maintenance regimen was mandatory. Immunosuppressants were suggested to be more effective in patients with worsening (i.e., with worsening of dyspnea and/or pulmonary functional tests) SSc-ILD. We aimed to assess the efficacy of mycophenolate mofetil (MMF) as a maintenance regimen after CYC in worsening SSc-ILD. Methods All patients (n = 20) with worsening SSc-ILD were retrospectively included. Treatment consisted of 6 to 12 monthly pulses of CYC followed by MMF and response was assessed by the evolution of the forced vital capacity (FVC) and carbon monoxide diffusing capacity (DLCO) during follow-up. Results At the end of CYC pulses, SSc-ILD had improved in 7 (35%) patients, stabilized in 10 (50%) (i.e., 85% of responders) and worsened in 3 (15%) when compared to baseline. After 6 months of MMF, 70% were still responders while 30% had worsened when compared to baseline (i.e., before CYC). After 12 months on MMF, 55% were responders and 45% had worsened when compared to baseline. Evolution of the FVC slope significantly improved on CYC as well as on MMF. Conclusions A strategy combining IV CYC followed by maintenance MMF for worsening SSc-ILD was associated with stabilization or improvement of pulmonary function tests in only 55% of patients after 12 months of MMF. This suggests that careful monitoring for worsening is mandatory during MMF maintenance and that improvement in managing worsening SSc-ILD is still needed.