Determinants of survival in idiopathic pulmonary fibrosis.

Abstract

To identify the determinants of survival in patients with idiopathic pulmonary fibrosis (IPF), we performed a survival analysis on 74 subjects with IPF. The study subjects were on average 64 yr of age (range, 25 to 83 yr), 62% were male, and 29% were never smokers. A tissue diagnosis was made in 67 (91%) of our study subjects. These subjects were followed for a mean period of 4 yr (range, 1.4 to 118.8 months) after the onset of pulmonary symptoms. During the period of observation, 41 subjects died (median survival = 28.2 months) and 33 continue to survive (median follow-up period = 60.9 months). A univariate analysis demonstrated that diminished survival was significantly associated with male gender (hazard ratio = 1.98; 95% confidence interval [CI] = 1.01-3.85), a higher FEV1/FVC ratio (hazard ratio = 1.82 [per 10% increase in the FEV1/FVC ratio]; 95% CI = 1.21-2.73), a lower percent predicted FVC (hazard ratio = 0.74; 95% CI = 0.60-0.91), a lower percent predicted total lung capacity (TLC) (hazard ratio = 0.75; 95% CI = 0.60-0.94), a lower percent predicted diffusing capacity of carbon monoxide (DLCO) (hazard ratio = 0.69; 95% CI = 0.53-0.89), a higher ILO profusion category on chest radiograph (hazard ratio = 3.52; 95% CI = 1.58-7.87), and an enhanced release of prostaglandin E2 (PGE2) by cultured alveolar macrophages (hazard ratio = 1.32 [per 10 pm/ml of PGE2]; 95% CI = 1.07-1.62).(ABSTRACT TRUNCATED AT 250 WORDS)

The timed walk test as a measure of severity and survival in idiopathic pulmonary fibrosis

“…The DL,CO, Pa,O 2 and PA-a,O 2 difference, which are strongly associated with the walk velocity and end-exercise saturation in this study, reflect the severity of parenchymal abnormalities in IPF and have consistently been associated with clinically important endpoints [7,8,12,[28][29][30][31]. There was no clear association between the TWT and lung volumes in this study.…”

“…Prognostic factors that have been variably associated with survival include age [6], smoking status [7], sex [8], resting pulmonary function [9], histopathology score [7], fibrotic score based on high-resolution computed tomography [10,11], and initial response to treatment with corticosteroids [10]. A composite score of clinical, radiographical and physiological variables has been associated with survival in IPF [12].…”

Nuclear Factor–Erythroid-2–Related Factor 2 in Aging and Lung Fibrosis

“…105 IPF is the most fatal of the interstitial lung diseases, with a median survival of <3 years. 106 The role of aging in IPF is incompletely understood. Exposure of lungs to various endogenous and exogenous oxidants may elicit inflammatory and fibrotic responses, leading to impaired antioxidant capacity.…”

Long‐Term Outcome and Use of 6‐Minute Walk Test in West Highland White Terriers with Idiopathic Pulmonary Fibrosis

“…In human IPF, the search for prognostic factors has been extensive as the course of the disease can vary greatly among patients, from rapidly to slowly progressive to a step‐like process 6. Several factors have been associated with poor survival in humans, including decreased walking distance in 6‐minute walk test (6MWT),8 increasing grade of interstitial fibrosis on thoracic radiographs,9 fibrosis score and traction bronchiectasis in thin‐section computed tomography,10 increased bronchoalveolar lavage (BAL) neutrophilia,11 and presence of pulmonary hypertension 12. In human IPF with a step‐like progression, periods of relative stability are interrupted by acute exacerbations (AEs), which are associated with high mortality and a histopathologic pattern of diffuse alveolar damage (DAD) 6.…”