Objective To investigate the clinical characteristics of fibrosing interstitial lung diseases, and explore its risk factors of mortality and of developing a progressive phenotype. Methods A total of 58 patients who suffered from fibrosing interstitial lung diseases admitted to the Chinese PLA General Hospital during May and December 2019 were prospectively enrolled in this study. Their clinical data were collected. And chest CT scanning, lung function test and 6-minute walk test(6MWT) were performed. The dyspnea score was assessed with the University of California, San Diego shortness of breath questionnaire (SOBQ). The patients were followed up for 1 year to evaluate the condition. SPSS statistics 19.0 and R 3.6.1 were used to perform the statistical analysis. Student′s t test or Chi-square test was employed for intergroup comparison for different data types. Competing risk model and Fine-Gray competing risk regression model were adopted to extract the risk factors of progressive fibrosis and mortality. Results Of the 58 patients, 35 (60.34%) were diagnosed as idiopathic pulmonary fibrosis (IPF), 14 (24.14%) as interstitial lung disease associated with connective tissue diseases, and 9 (15.52%) as pulmonary fibrosis caused by other causes. Twelve cases (20.69%) developed into progressive fibrotic phenotype, and 8 (13.79%) died, including 6 due to acute exacerbation of fibrosis. Univariate analysis showed that the risk factors for the development of progressive fibrotic phenotype were diagnosis of IPF, high reticular score and honeycombing score in high resolution CT (HRCT) scans, and the risk factors for death were short 6-minute walk distance (the dead had a significantly higher ratio of shorter than 300 m) and higher SOBQ score. Multivariate analysis showed that high HRCT reticular score was the risk factor for progressive fibrotic phenotype (RC=0.687, HR=1.99,95%CI 1.03-3.85, P=0.042), and no risk factors for death were found. Conclusion Patients with IPF, high HRCT reticular and honeycombing scores are at a higher risk of developing progressive fibrotic phenotype. Those with 6-minute walk distance shorter than 300 m and higher SOBQ scores are prone to death.