Retrospective evaluation of treatment outcomes in connective tissue disease associated interstitial lung disease

CTD associated ILD is a common complication, resulting in significant mortality and morbidity. The five-year survival rate of ILD patterns is 36.6% in UIP and 93.8% in NSIP. There are not sufficient studies to guide the management of BDH-ILD treatment. The only disease group in which randomized controlled study is performed is SSC-related ILD. The most preferred treatments are corticosteroids, immunosuppressive agents such as cyclophosphamide, azathioprine, and MMF. The use of the CD20 monoclonal antibody rituximab in BDH-ILD is becoming increasingly widespread. In this study, the results of treatment of patients with CTD-ILD were evaluated retrospectively , outcomes of patients treated with MMF and rituximab were analyzed. The mean duration of treatment was 28.5 months and 19.7 months. There was a statistically significant improvement in mean FVC and FEV1 in patients who received MMF therapy (p = 0.029 and p = 0.019), although an increase was also observed in DLCO but not statistically significant. With rituximab treatment, there was a decrease in FEV1 with no significant increase in FVC and FEV1. In 13 of the MMF-treated patients, there was no significant difference in HRCT findings before and after treatment, 1 patient showed progression and 1 patient had regression. There was no significant difference in HRCT findings of 7 patients receiving rituximab, 2 patients showed progression and 1 patient had regression. Majority of patients in the rituximab arm were diagnosed with RA-UIP and there is no significant improvement in tihs group. We emphasize that MMF therapy may have a positive impact on CTD-ILD prognosis. There is a need to examine the efficacy of MMF with more prospective randomised controlled trials in larger patient groups.