Abstract

Introduction: Childhood interstitial lung disease (chILD) lacks randomized controlled trials and management guidelines. Empirical treatments include oral prednisolone, pulse MPZ, and hydroxychloroquine, but their effectiveness remains uncertain.
 Aims and Objectives: To assess in a multidimensional manner the response of chILD patients to oral prednisolone, pulse MPZ and hydroxychloroquine treatment.
Methods: This retrospective cohort study included patients with chILD, who were followed up between 2010 and 2022.We compared the Fan Score before and after treatment to evaluate their clinical status. Physiological changes were assessed using FVC% and the 6-minute walk test (6mwt).
Results: 86 patients were included in the study, and 52 (60.4%) of them had received these treatments. A significant decrease was observed in the Fan Scores ( median :4 to 2,p=0.002). Patients had pre and post-6mwt and showed significant improvement (median 398m to 511m, p=0.026). There were no statistically significant differences between the FVC values; it was noted that the median FVC% value was increased from 59 to 64 (p=0.909). There was no significant change in the CT reports in terms of hyperinflation (p=0.655), ground glass appearance (p=0.109), pleural findings (p=0.739), lung nodules (p=0.317), emphysema or cyst appearance (p=0.705), atelectasis(p=0.763), septal/interstitial thickening (p=0.564) or consolidation(p=0.157).
Conclusion: These treatments may improve clinical conditions except pulmonary function tests and radiological imaging tests. Therefore, these treatments may not be enough to manage chILD completely.