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Introduction: The GAP model was widely used as a simple risk "screening" method for patients with idiopathic pulmonary fibrosis (IPF). Objectives: We sought to validate the GAP model in Chinese patients with IPF to evaluate whether it can accurately predict the risk for mortality. Methods: A total of 212 patients with IPF diagnosed at China‐Japan Friendship Hospital from 2015 to 2019 were enrolled. The latest follow‐up ended in September 2022. Cumulative mortality of each GAP stage was calculated and compared based on Fine‐Gray models for survival, and lung transplantation was treated as a competing risk. The performance of the model was evaluated in terms of both discrimination and calibration. Results: The cumulative mortality in patients with GAP stage III was significantly higher than that in those with GAP stage I or II (Gray's test p < 0.0001). The Harrell c‐index for the GAP calculator was 0.736 (95% CI: 0.667–0.864). The discrimination for the GAP staging system were similar with that for the GAP calculator. The GAP model overestimated the mortality rate at 1‐ and 2‐year in patients classified as GAP stage I (6.90% vs. 1.77% for 1‐year, 14.20% vs. 6.78% for 2‐year). Conclusions: Our findings indicated that the GAP model overestimated the mortality rate in mild group. [ABSTRACT FROM AUTHOR]

Background: Serum Krebs von den Lungen‐6 (KL‐6) has been reported to be elevated in patients with idiopathic pulmonary fibrosis (IPF). Objective: The aim of this study was to evaluate the diagnostic value of KL‐6 and whether the expression value of KL‐6 could indicate the severity of the disease in IPF patients. To address this question, it is necessary to see whether the patients' physical characteristics and other clinical conditions could affect the baseline KL‐6 level. Design We conducted a study of 100 patients who were diagnosed with IPF. Lung function, computed tomography (CT), and serological lab tests data were analyzed. Results: The tests showed that there is a significant elevation of KL‐6 in IPF patients compared with other interstitial lung disease (ILD) and healthy controls. It was noted that serum KL‐6 is a stable biomarker not affected by lung infection and smoking, though IPF patients with antinuclear antibody (ANA) showed higher KL‐6 levels. KL‐6, in conjunction with poor pulmonary function and higher radiological fibrosis scores, indicates the severity of the disease but not poor survival. Conclusions: It is identified that serum KL‐6 is a useful noninvasive biomarker to help improve the certainty of IPF diagnosis from other interstitial lung disease and assist evaluation of disease severity and prognosis. [ABSTRACT FROM AUTHOR]