Outcomes After Hospitalization in Idiopathic Pulmonary Fibrosis

Brown, Anne; Fischer, Chelsea P.; Shlobin, Oksana A.; Buhr, Russell G.; Ahmad, Shahzad; Weir, Nargues; Nathan, Steven D.

doi:10.1378/chest.13-2424

Cited by 77 publications

(102 citation statements)

References 30 publications

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“…Non-elective hospitalisations are associated with considerable cost [63], and respiratory-related hospitalisations in particular have prognostic significance in IPF [64][65][66]. BROWN et al [65] have reported that most hospitalisations in IPF are respiratory-related, and associated with high in-hospital mortality and limited survival beyond discharge.…”

Section: Importance Of Non-elective Hospitalisationmentioning

confidence: 99%

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Recent lessons learned in the management of acute exacerbation of idiopathic pulmonary fibrosis

2017

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Recognising recent advances, the definition and diagnostic criteria for acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) have been updated by an international working group. The new definition describes any acute, clinically significant respiratory deterioration (both idiopathic and triggered events) characterised by evidence of new widespread alveolar abnormality. The new criteria require a previous or concurrent diagnosis of IPF, an acute worsening or development of dyspnoea typically less than 1 month in duration, chest imaging evidence on computed tomography (CT) of new bilateral ground-glass opacity and/or consolidation superimposed on a background imaging pattern of usual interstitial pneumonia not fully explained by cardiac failure or fluid overload. Due to high in-hospital mortality rates, current treatment guidelines say that the majority of patients with AE-IPF should not receive mechanical ventilation. However, new data suggest that the prognosis may have improved. This modest improvement in overall survival seen in more recent studies may be the result of differences in the diagnostic criteria, study design, baseline clinical risk factors and/or improvements in management. Based on our updated knowledge of possible preventive and therapeutic measures, including mechanical ventilation and pharmacological therapies, the current approach to the treatment of AE-IPF requires careful decision-making.

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Section: Importance Of Non-elective Hospitalisationmentioning

confidence: 99%

“…BROWN et al [65] have reported that most hospitalisations in IPF are respiratory-related, and associated with high in-hospital mortality and limited survival beyond discharge. Even in the setting of a respiratory-related hospitalisation, the presence of AE is associated with a worse prognosis.…”

Section: Importance Of Non-elective Hospitalisationmentioning

confidence: 99%

Recent lessons learned in the management of acute exacerbation of idiopathic pulmonary fibrosis

2017

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“…Prior studies have demonstrated that, when considered individually, both acute hospitalizations 17, 20 and decline in FVC 7, 12–5 are associated with decreased survival in IPF. Interestingly, in our study there was little overlap (10%) between patients with early hospitalization and early disease progression; in fact, patients who experienced one surrogate event were more likely not to experience the other, a finding that did not appear to be explained by missing FVC data.…”

Section: Discussionmentioning

confidence: 99%

“…9 A recent report has suggested that hospitalizations are independently associated with decreased survival in IPF, and that respiratory hospitalizations portend especially poor long-term outcomes. 20 …”

Section: Introductionmentioning

confidence: 99%

Association of hospital admission and forced vital capacity endpoints with survival in patients with idiopathic pulmonary fibrosis: analysis of a pooled cohort from three clinical trials

Durheim

Collard

Roberts

et al. 2015

The Lancet Respiratory Medicine

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Background Mortality is an impractical primary endpoint for idiopathic pulmonary fibrosis (IPF) clinical trials in patients with mild to moderate physiological impairment due to low event rates. Change in forced vital capacity (FVC) is widely accepted as a surrogate for mortality in patients with IPF, and has been the most common primary endpoint in IPF clinical trials. The role of hospitalization as a predictor for mortality independent of FVC decline has not been well defined. In a pooled cohort of subjects with IPF, we determined the independent and combined association of hospitalization and a categorical 10% decrease in FVC with all-cause mortality. Methods The incidence of non-elective hospitalization and 10% decrease in FVC was compared across strata of baseline physiologic impairment. Cox proportional hazards models were used to evaluate the risk of all-cause mortality associated with hospitalizations and 10% decrease in FVC occurring up to a pre-defined time point. Findings Hospitalization and categorical 10% decrease in FVC occurred frequently across strata of baseline physiologic impairment. The majority of hospitalized patients did not have a 10% decrease in FVC, a finding that was not explained by missed spirometry visits. Both surrogate events were associated with subsequent time to death from any cause. When causes of hospitalizations were considered, only respiratory hospitalizations remained associated with mortality. Interpretation Hospitalizations may be appropriate components of a clinically meaningful composite endpoint that improves feasibility in IPF clinical trials. Funding National Heart, Lung, and Blood Institute (NHLBI) and The Cowl in Family Fund at the Chicago Community Trust.

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“…It would also allow for cohort enrichment in clinical trials designed around primary endpoints of disease progression (7). Disease progression in IPF is most commonly defined by decline in FVC (6,(8)(9)(10)(11), but there are other definitions, including worsening symptoms (e.g., worsening dyspnea) (12,13), worsening physical function (14)(15)(16), and the occurrence of acute respiratory worsening requiring hospitalization (6,9,17,18). All of these measures of disease progression have been associated with increased risk of subsequent death in patients with IPF.…”

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confidence: 99%

Predictors of Mortality Poorly Predict Common Measures of Disease Progression in Idiopathic Pulmonary Fibrosis

Ley¹,

Bradford²,

Vittinghoff

et al. 2016

Am J Respir Crit Care Med

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Rationale: Mortality prediction is well studied in idiopathic pulmonary fibrosis (IPF), but little is known about predictors of premortality disease progression. Identification of patients at risk for disease progression would be useful for clinical decision-making and designing clinical trials.Objectives: To develop prediction models for disease progression in IPF.Methods: In a large clinical trial cohort of patients with IPF (n = 1,113), we comprehensively screened multivariate models of candidate baseline and past-change predictors for disease progression defined by 48-week worsening of FVC, dyspnea (University of California, San Diego Shortness of Breath Questionnaire [UCSD SOBQ]), 6-minute-walk distance (6MWD), and occurrence of respiratory hospitalization, or death. Progression outcomes were modeled as appropriate, by slope change using linear regression models and time to binary outcomes using Cox proportional hazards models. Measurements and Main Results:The overall cohort experienced considerable disease progression. Top-performing prediction models did not meaningfully predict most measures of disease progression. For example, prediction modeling explained less than or equal to 1% of the observed variation in 48-week slope change in FVC, UCSD SOBQ, and 6MWD. Models performed better for binary measures of time to disease progression but were still largely inaccurate (cross-validated C statistic <0.63 for >10% decline in FVC or death, <0.68 for >20-U increase in UCSD SOBQ or death, <0.70 for >100 m decline in 6MWD or death). Models for time to respiratory hospitalization or death (C statistic <0.77) or death alone (C statistic <0.81) demonstrated acceptable discriminative performance.Conclusions: Clinical prediction models poorly predicted physiologic and functional disease progression in IPF. This is in contrast to respiratory hospitalization and mortality prediction.

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Outcomes After Hospitalization in Idiopathic Pulmonary Fibrosis

Cited by 77 publications

References 30 publications

Recent lessons learned in the management of acute exacerbation of idiopathic pulmonary fibrosis

Recent lessons learned in the management of acute exacerbation of idiopathic pulmonary fibrosis

Association of hospital admission and forced vital capacity endpoints with survival in patients with idiopathic pulmonary fibrosis: analysis of a pooled cohort from three clinical trials

Predictors of Mortality Poorly Predict Common Measures of Disease Progression in Idiopathic Pulmonary Fibrosis

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