The natural history of @entity2416 ( @entity2416 ) 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 @entity1 . 70 @entity1 newly diagnosed with @entity2416 were prospectively followed for at least 3 yrs. Baseline evaluation included Medical Research Council dyspnoea score (MRCDS), 6-min walk test, pulmonary function tests, all of which were repeated at 6 months, and high-resolution computed tomography. A retrospective cohort of 68 @entity1 was used for confirmation. Mean survival from the time of diagnosis was 30 months, with a 3-yr mortality of 46%. A Risk stratificatiOn ScorE (ROSE) based on MRCDS > 3, 6-min walking distance  <= 72% predicted and composite physiologic index > 41 predicted 3-yr mortality with high specificity. 6-month progression of ROSE predicted rapid progression. 3-yr incidence of AE was 18.6%, mostly occurring in the first 18 months; risk factors for AE were concomitant @entity2415 and low diffusing coefficient of the lung for @entity1223 . Results were confirmed in an independent cohort of @entity1 . In newly diagnosed @entity2416 , advanced disease at presentation, rapid progression and AEs are the determinants of 3-yr survival. The purpose of the multifactorial ROSE is to risk-stratify @entity1 in order to predict survival and detect rapid disease progression.

Predicting survival in newly diagnosed XXXX : a 3-year prospective study.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity2416 :: ('MESH:D054990', 'Disease') :: ['IPF', 'idiopathic pulmonary fibrosis'] @entity1223 :: ('MESH:D002248', 'Chemical') :: ['carbon monoxide'] @entity2415 :: ('MESH:D004646', 'Disease') :: ['emphysema']
@entity2416 :: (MESH:D054990,Disease) :: ['idiopathic pulmonary fibrosis']
