Ascertainment of Individual Risk of Mortality for Patients with Idiopathic Pulmonary Fibrosis

Abstract

Rationale: Several predictors of mortality in patients with idiopathic pulmonary fibrosis have been described; however, there is a need for a practical and accurate method of quantifying the prognosis of individual patients. Objectives: Develop a practical mortality risk scoring system for patients with idiopathic pulmonary fibrosis. Methods: We used a Cox proportional hazards model and data from two clinical trials (n ¼ 1,099) to identify independent predictors of 1-year mortality among patients with idiopathic pulmonary fibrosis. From the comprehensive model, an abbreviated clinical model comprised of only those predictors that are readily and reliably ascertained by clinicians was derived. Beta coefficients for each predictor were then used to develop a practical mortality risk scoring system. Measurements and Main Results: Independent predictors of mortality included age, respiratory hospitalization, percent predicted FVC, 24-week change in FVC, percent predicted carbon monoxide diffusing capacity, 24-week change in percent predicted carbon monoxide diffusing capacity, and 24-week change in health-related quality of life. An abbreviated clinical model comprising only four predictors (age, respiratory hospitalization, percent predicted FVC, and 24-wk change in FVC), and the corresponding risk scoring system produced estimates of 1-year mortality risk consistent with observed data (9.9% vs. 9.7%; C statistic ¼ 0.75; 95% confidence interval, 0.71-0.79). Conclusions:The prognosis for patients with idiopathic pulmonary fibrosis may be accurately determined using four readily ascertainable predictors. Our simplified scoring system may be a valuable tool for determining prognosis and guiding clinical management. Additional research is needed to validate the applicability and accuracy of the scoring system.

Staging of idiopathic pulmonary fibrosis: past, present and future

“…the total score for a patient aged 70 years, with no history of respiratory hospitalisation, FVC 51-65% predicted, and a 24-week change in FVC of -5 to -9.9% predicted is 8+0+13+10531. Reproduced with modification from [21] with permission from the publisher. .80…”

“…a patient with a total risk score of 31 has a predicted 1-year probability of death of 20-30%. Reproduced with modification from [21] with permission from the publisher. [14][15][16][17][18][19][20].…”

“…Using a large clinical trial population of patients with IPF, DU BOIS et al [21] developed a scoring system comprised of four predictors: age, recent respiratory hospitalisation, baseline FVC and 24-week change in FVC. As shown in table 1, the sum of individual scores for the four parameters are used to obtain a composite score that can accurately predict the 1-year risk of death (table 2).…”

Challenges in idiopathic pulmonary fibrosis trials: the point on end-points

“…A mortality risk scoring model for patients with 1PF was recently provided by DU BOIS et al [35]. The proposed abbreviated clinical model comprises four readily available clinical measures of disease status (age, FVC at baseline, 24-week change in FVC and 24-week history of respiratory hospitalisation) that reliably predict 1-yr mortality in patients with IPF.…”

“…The proposed abbreviated clinical model comprises four readily available clinical measures of disease status (age, FVC at baseline, 24-week change in FVC and 24-week history of respiratory hospitalisation) that reliably predict 1-yr mortality in patients with IPF. This simplified scoring system may be a valuable tool for determining mortality risk of IPF patients in clinical practice, facilitating clinical decision making in the management of patients with IPF [35].…”