Idiopathic Pulmonary Fibrosis in United States Automated Claims. Incidence, Prevalence, and Algorithm Validation

Esposito, Daina B.; Lanes, Stephan; Donneyong, Macarius; Holick, Crystal N.; Lasky, Joseph A.; Lederer, David J.; Nathan, Steven D.; O’Quinn, Sean; Parker, J.D.; Tran, Trung

doi:10.1164/rccm.201504-0818oc

Cited by 112 publications

(87 citation statements)

References 25 publications

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“…They found no significant difference in the distribution of patient demographics and clinical characteristics between the subsample and the database population [23], thus validating the proper selection of IPF patients using this code. Very recently, a study presented another method to assess the performance of algorithms applied to identify IPF patients including validation by calculating positive predictive value using medical records [27]. This approach, which may be more accurate, could not be applied in our study, due to the difficulty of obtaining nominative patient-level data in France.…”

Section: Discussionmentioning

confidence: 99%

Burden of Idiopathic Pulmonary Fibrosis Progression: A 5-Year Longitudinal Follow-Up Study

Cottin

Schmidt²,

Laura³

et al. 2017

PLoS ONE

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Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease with an unpredictable course. An observational study was set up using the French hospital discharge database to describe the reasons, outcomes and costs of hospitalisations related to this disease. Patients newly hospitalised for idiopathic pulmonary fibrosis (ICD-10 code: J84.1) in 2008 were identified and followed for 5 years. As J84.1 includes other fibrotic pulmonary diseases, an algorithm excluding age<50 years and presence of a differential diagnosis in the following year was defined. Overall, 6,476 patients were identified; of whom 30% were admitted through the emergency unit and 12% died during their first hospitalisation. Most of patients were hospitalised at least once for one or several acute events (n = 5,635; 87.0% of patients), of whom 36.5% of patients with an acute respiratory worsening (in-hospital mortality of 17.0% and median cost of €3,224; interquartile range (IQR €889–6,092)), 43.7% of patients with a respiratory infection (in-hospital mortality of 29.5% and median cost of €5,432 (IQR, €3,620–9,115)) and 51.7% of patients with a cardiac event (in-hospital mortality of 35.7% and median cost of €4,584 (IQR, €2,803–6,399)); 30.2% of these events occurred during the first hospitalisation. Finally, the 3-year in-hospital mortality crude rate was 36.8%. This study is the first providing extensive data on hospitalisations in patients with pulmonary fibrosis, mostly idiopathic, in France, demonstrating high burden and hospital cost.

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Section: Discussionmentioning

confidence: 99%

Burden of Idiopathic Pulmonary Fibrosis Progression: A 5-Year Longitudinal Follow-Up Study

Cottin

Schmidt²,

Laura³

et al. 2017

PLoS ONE

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“…First, there is debate on how to identify IPF patients using claims data. The ICD-9-CM code we used has been used before in several publications [1, 6, 23, 24], however a recent validation study (not in the NIS) found it had a positive predictive value of 30-60% [29]. While less than desirable, this positive predictive value is within the range reported in study of ICD-9-CM codes for 32 conditions (median 80.7%, mean 77%, range 23-100%) [30].…”

Section: Discussionmentioning

confidence: 99%

Mechanical ventilation in idiopathic pulmonary fibrosis: a nationwide analysis of ventilator use, outcomes, and resource burden

Mooney

Raimundo²,

Chang

et al. 2017

BMC Pulm Med

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BackgroundIdiopathic pulmonary fibrosis (IPF) is associated with increased risk of respiratory-related hospitalizations. Studies suggest mechanical ventilation (MV) use in IPF does not improve outcomes and guidelines recommend against its general use. Our objective was to investigate MV use and association with cost and mortality in IPF.MethodsThis retrospective study, using a nationwide sample, included claims with IPF (ICD-9-CM: 516.3) in 2009–2011 and principal respiratory disease diagnosis (ICD-9-CM: 460–519); excluding lung transplant. Regression models were used to determine predictors of MV and association with cost, LOS, and mortality. Domain analysis was used to account for use of subpopulation. Costs were adjusted to 2011. Data on patient severity not available.ResultsTwenty two thousand three hundred fifty non-transplant IPF patients were admitted with principal respiratory disease diagnosis: Mean age 70.0 (SD 13.9), 49.1% female, mean LOS 7.4 (SD 8.2). MV was used in 11.4% of patients with a non-significant decline over time. In regression models, MV was associated with an increased stay of 9.78 days (95% CI 8.38–11.18) and increased cost of $36,583 (95% CI $32,021–41,147). MV users had significantly increased mortality (OR 15.55, 95% CI 12.13–19.95) versus nonusers.ConclusionsMechanical ventilation use has not significantly changed over time and is mostly used in younger patients and those admitted for non-IPF respiratory conditions. MV was associated with a 4-fold admission cost increase ($49,924 versus $11,742) and a 7-fold mortality increase (56% versus 7.5%), although patients who receive MV may differ from those who do not. Advances in treatment and decision aids are needed to improve outcomes in IPF.

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“…Epidemiological data for IPF can vary widely due to variations in the data collection methods and classification terms used (table 1) [11,12]. Throughout Europe and North America, the estimated incidence of IPF has been reported to range between 2.8 and 19 cases per 100 000 people per year [23][24][25].…”

Section: Introductionmentioning

confidence: 99%

The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype

et al. 2018

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@ERSpublications ILDs with a progressive-fibrosing phenotype appear to be more common in older adults and are associated with a complex network of environmental and genetic factors. Further epidemiological studies are warranted to help identify these patients. http://ow.ly/SY6m30mWytM Cite this article as: Olson AL, Gifford AH, Inase N, et al. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev 2018; 27: 180077 [https://doi.ABSTRACT The availability of epidemiological data relating to interstitial lung diseases (ILDs) has increased over recent years, but information on the prevalence and incidence of ILDs of different aetiologies remains limited. Despite global distribution, the proportion of patients who develop a progressive phenotype across different ILDs is not well known. Disease behaviour is well documented in idiopathic pulmonary fibrosis but idiosyncratic in other ILDs that may present a progressive fibrosing phenotype. Possible reasons may include the heterogeneous nature of the aetiology, the complexity of diagnosis (and subsequent documentation of cases) and the methods employed to retrospectively analyse patient databases. This review presents a broad overview of the epidemiological data available for ILDs that may present a progressive-fibrosing phenotype, collectively and stratified according to clinical classification. We also note where further data are needed in comparison to the well-studied IPF indication.

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Idiopathic Pulmonary Fibrosis in United States Automated Claims. Incidence, Prevalence, and Algorithm Validation

Abstract: Sensitive algorithms without correction for false positive errors overestimated incidence and prevalence of IPF. An IPF score offered the greatest PPV, but it requires further validation.

Cited by 112 publications

References 25 publications

Burden of Idiopathic Pulmonary Fibrosis Progression: A 5-Year Longitudinal Follow-Up Study

Burden of Idiopathic Pulmonary Fibrosis Progression: A 5-Year Longitudinal Follow-Up Study

Mechanical ventilation in idiopathic pulmonary fibrosis: a nationwide analysis of ventilator use, outcomes, and resource burden

The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype

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