Fibrosing interstitial lung disease (ILD) can cause high mortality and sensitive evaluation of fibrosing ILD could be critical. The aim of this study is to develop a scoring system to predict prognosis of fibrosing ILD. 339 patients with fibrosing ILD were enrolled as a derivation cohort. Cox multiple regression analysis indicated that smoking history (HR = 3.826, p = 0.001), age(HR = 1.043, p = 0.015), CEA(HR = 1.059, p = 0.049),CYFRA21-1(HR = 1.177, p = 0.004) and DLCO% predicted (HR = 0.979, p = 0.032) were independent prognostic factors for fibrosing ILD. The clinical scoring system for fibrosing ILD was established based on the clinical variables (age [A], CEA and CYFRA21-1 [C], DLCO% predicted [D], and smoking history [S]; ACDS). The area under the receiver operating characteristic curve (AUROC) of the scoring system for predicting prognosis of fibrosing ILD was 0.90 (95%CI: 0.87-0.94, p < 0.001). The cutoff value was 2.5 with their corresponding specificity (90.7%) and sensitivity (78.8%). To validate the value of ACDS score levels to predict the survival of patients with fibrosing ILD, 98 additional fibrosing ILD patients were included as a validation cohort. The log-rank test showed a significant difference in survival between the two groups(ACDS score < 2.5 and ACDS score ≥ 2.5) in validation cohort. The independent risk factors for mortality in patients with fibrosing ILD are higher CEA, higher CYFRA21-1, smoking history, lower DLCO%predicted at baseline and older age. ACDS is a simple and feasible clinical model for predicting survival of fibrosing ILD.
© 2022. The Author(s).