PURPOSE: Pulmonary fibrosis encompasses a group of diffuse parenchymal lung diseases with multiple causes and variable prognosis. The median survival for the idiopathic type is grim: 3-5 years. Due to the rarity of the disease, survival across age, race, and gender is not well-described. This study uses a large database to analyze survival in pulmonary fibrosis according to these variables.
METHODS: ICD-9 codes were used to identify cases of pulmonary fibrosis in the Veterans Affairs National Patient Care claims database between 2001 and 2007. Cases were included if they had one or more primary care visits in the year prior to diagnosis, and excluded if they had concurrent diagnosis of asbestosis or rheumatic disease. Survival analysis was performed with Kaplan-Meier curves for race, gender, and age categories. Log-rank tests were utilized to compare survival between groups.
RESULTS: 14,448 cases of pulmonary fibrosis were identified. Median survival was described according to the following age categories: a. Ages 50 and younger (n=687): median survival was greater than 8.0 years, b. Ages 51-60 (n=2353): median survival 7.5 years (95% confidence interval (CI) 7.0-8.1), d. Ages 61-70 (n=3276): median survival 4.6 years (CI 4.4-4.8), e. Ages 71-80 (n=5682): median survival 3.2 years (CI 3.1-3.4), and f. Greater than 80 years (n=2450): median survival 2.6 years (CI 2.5-2.8). Females had increased overall survival as compared to men (6.2 years (CI 5.2-9.0) vs. 3.9 years (CI 3.8-4.0), p<0.0001) and this effect was more pronounced with increasing age. Although African-Americans appeared to have better overall survival (p=0.004), this was related to a younger age of diagnosis. There was no difference in survival between race categories when stratified by age.
CONCLUSION: Survival with pulmonary fibrosis is affected by age and gender in the Veterans population. Younger patients and females have increased survival compared to older and male patients. Race does not affect survival when taking age into account.
CLINICAL IMPLICATIONS: Survival related to demographics should be considered when counseling patients with pulmonary fibrosis about prognosis.
DISCLOSURE: Alicia Gerke, No Financial Disclosure Information; No Product/Research Disclosure Information