Development of a scoring system with multidimensional markers for fibrosing interstitial lung disease.

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.