Abstract
The natural history of idiopathic pulmonary fibrosis (IPF) is not well defined and its clinical course is variable. We sought to investigate the survival and incidence of acute exacerbations (AEs) and their significant predictors in newly diagnosed patients.
Seventy patients newly diagnosed with IPF were prospectively followed for at least 3 years. Baseline evaluation included MRC dyspnoea score (MRCDS), 6-min walk test, pulmonary function tests, which were all repeated at 6 months, and HRCT. A retrospective cohort of 68 patients was used for confirmation.
Mean survival from the time of diagnosis was 30 months, with a 3-year mortality of 46%. A Risk StratificatiOn ScorE (ROSE) based on MRCDS>3, 6-min walking distance≤72% pred and composite physiologic index >41 predicted 3-year mortality with high specificity. 6-month progression of ROSE predicted rapid progression. 3-year incidence of AE was 18.6%, mostly occurring in the first 18 months; risk factors for AE were concomitant emphysema and low DL,CO. Results were confirmed in an independent cohort of patients.
In newly diagnosed IPF, advanced disease at presentation, rapid progression and AEs are the determinants of 3-year survival. The purpose of the multifactorial ROSE is to risk-stratify patients in order to predict survival and detect rapid disease progression.
- ERS