Abstract Background Though connective tissue disease-associated interstitial lung disease (CTD-ILD) is a heterogeneous group, the treatment strategy in immunosuppressant selection is summarily consistent across all CTD-ILD subtypes. Our study aimed to assess the effectiveness and safety of different treatment options, including both monotherapy and combination regimens, in patients with CTD-ILD. Methods We conducted a dual-center retrospective, observational cohort study. Patients with various CTD-ILD were enrolled from January 2022 to September 2024 and followed up for 12 months. Untreated patients formed the control group. Treatment groups included glucocorticoids (GCS), GCS + mycophenolate mofetil (MMF), GCS + cyclophosphamide (CYC), and GCS+MMF+CYC. The primary outcome was the change from baseline in the percent predicted forced vital capacity (FVC %pred) at 6 and 12 months. Secondary outcomes included changes from baseline in FVC absolute value, diffusion lung capacity for CO (DLco) %pred, the percentage of radiological interstitial patterns including ground-glass opacities (GGO) and fibrosis (i.e. reticulation and honeycombing) at 6 and 12 months, adverse event rates and infection rates at 12 months. Results Data from 178 patients with CTD-ILD were included in the final analysis. There were 17 in the untreated group, 37 in the GCS group, 81 in the GCS+MMF group, 25 in the GCS + CYC group, and 18 in the GCS + MMF + CYC group. Compared with baseline, the untreated group showed decreases in FVC %pred and FVC absolute value at both 6 and 12 months. In contrast, the GCS+MMF, GCS+CYC, and GCS+MMF+CYC groups showed increases in FVC %pred and FVC absolute value at 6 months, and the GCS+MMF and GCS+CYC groups also showed increases at 12 months. The percentage of fibrosis increased in the untreated group at 6 months and 12 months but remained stable in the treatment groups. GGO% and reticulation% decreased in the treatment groups at 6 months compared to baseline. There were no significant differences in adverse event rates and infection rates among the groups. Conclusions In patients with various CTD-ILD, different immunosuppressant combination therapies demonstrated similarly significant improvements in lung function and radiological interstitial patterns, with comparable adverse event and infection rates. This abstract is funded by: Shanghai Municipal Health Commission (No.20244Z0019), National Key Technologies R & D Program Precision Medicine Research (2024ZD0528901), Physician-scientist project of Shanghai Jiao Tong University School of Medicine (No.20240820)
Li et al. (Fri,) studied this question.