Effectiveness and safety of mycophenolate mofetil in connective tissue disorder associated interstitial lung disease: A real-life experience from India

Abstract

Aim: To study the effectiveness and safety of mycophenolate mofetil (MMF) in patients with connective tissue disease (CTD) associated interstitial lung disease (ILD). Methods: The retrospective observational study was carried out from Jan 2015 to Feb 2019. Symptomatic CTD-LD patients with (HRCT chest) documented ILD and abnormal pulmonary function test (forced vital capacity, FVC< 70%), who were treated with MMF were included. The treatment response was assessed clinically by pulmonary function test and radiology. Clinical assessment and PFT were done at baseline, 6, 12 and 24 months. HRCT chest was done at baseline and 24 months. Results and analysis: Out of 33 patients, 13 had MCTD, 12 had RA, and remaining 8 belonged to the systemic sclerosis (SSc)-predominant groups (3: diffuse cutaneous SSc, 2: SSc/myositis overlap, and 1 each with Sjogren’s syndrome, SLE/Sjogren’s overlap and interstitial pneumonia with autoimmune features). Increased female predominance was noted (90.9%). The mean FVC at baseline noted in MCTD, RA and SSc-predominant groups were 62±6.17, 64±4.17 and 59±6 respectively. Improvement was reported in 4 patients, each in MCTD and RA groups. Eight patients in the MCTD group and 7 each in RA and SSc- predominant groups had a stable lung disease. One patient each in all the groups reported worsening of the disease. There was a positive trend in FEV1 and FVC with treatment. No significant difference in FEV1 and FVC values with treatment was noted across the three groups. Numerical differences in the mean values of FEV1 and FVC between two groups (NSIP and UIP) were noted, but was not statistically significant. All the subjects completed 24 months of follow-up. They were on 2 g/day of MMF for the first 12 months, followed by a maintenance of 1.5- 2 g/day for the next 12 months. None of the subjects discontinued treatment due to intolerance or adverse effects.