Multicenter real-world data of antifibrotics in non-IPF progressive pulmonary fibrosis (PPF) patients

In this multicenter retrospective cohort study, we aimed to reveal the real-world data of PPF patients, and the effectiveness and reliability of AFs. Adult patients diagnosed with PPF and treated with AFs for at least 6 months were examined. A total of 222 patients were included in the study. Autoimmune disease-related ILD, fHP and fNSIP were the most common subtypes. Nintedanib (N) (70.3%) was utilized more than pirfenidone (P) (27.5%). The mean of FVC%, FVC (ml), DLCO% at the 0th, 6th and 12th months showed significant differences with temporal decreases [F(1.895, 217.75)=6.573, p=0.002, F(1.513, 160.341)=3.547, p=0.043 and F(1.741, 194.973)=3.383, p=0.002 respectively]. When the losses in the first 6 months and the second 6 months were compared, only DLCO% had a significant difference (p=0.014). The decrease in FVC values was stable. In PPF subgroup analysis; the radiological pattern, AF regimen, duration of treatment before AF, and AF duration after diagnosis had significant differences between groups (p=0.001, p=0.001, p=0.008, and p=0.014, respectively). Those under N treatment needed more additional treatment than the P group (67.3 vs 42.6%, p=0.001). While all PFT values had reduced more in the N group (p:0.013, 0.028, and 0.012, respectively), the P group, had a higher hospitalization rate and an overall longer duration of AF treatment (p=0.037 and p=0.001 respectively). There was no significant difference regarding clinical and radiological findings, and also PPF subgroups in terms of mortality. Only radiological progression was significantly associated with mortality (p=0.001). Conclusionally, AFs show consistent efficacy in PPF.