Burden Of Idiopathic Pulmonary Fibrosis Research Articles

The economic burden of idiopathic pulmonary fibrosis in Australia: a cost of illness study.

Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease found mostly in elderly persons, characterized by a high symptom burden and frequent encounters with health services. This study aimed to quantify the economic burden of IPF in Australia with a focus on resource utilization and associated direct costs. Participants were recruited from the Australian IPF Registry (AIPFR) between August 2018 and December 2019. Data on resource utilization and costs were collected via cost diaries and linked administrative data. Clinical data were collected from the AIPFR. A "bottom up" costing methodology was utilized, and the costing was performed from a partial societal perspective focusing primarily on direct medical and non-medical costs. Costs were standardized to 2021 Australian dollars ($). The average annual total direct costs per person with IPF was $31,655 (95% confidence interval (95% CI): $27,723-$35,757). Extrapolating costs based on prevalence estimates, the total annual costs in Australia are projected to be $299 million (95% CI: $262 million-$338 million). Costs were mainly driven by antifibrotic medication, hospital admissions and medications for comorbidities. Disease severity, comorbidities and antifibrotic medication all had varying impacts on resource utilization and costs. This cost-of-illness study provides the first comprehensive assessment of IPF-related direct costs in Australia, identifies the key cost drivers and provides a framework for future health economic analyses. Additionally, it provided insight into the major cost drivers which include antifibrotic medication, hospital admissions and medications related to comorbidities. Our findings emphasize the importance of the appropriate management of comorbidities in the care of people with IPF as this was one of the main reasons for hospitalizations.

Mortality Associated with Idiopathic Pulmonary Fibrosis in Northeastern Italy, 2008-2020: A Multiple Cause of Death Analysis.

Mortality from idiopathic pulmonary fibrosis (IPF) is increasing in most European countries, but there are no data for Italy. We analysed the registry data from a region in northeastern Italy to assess the trends in IPF-related mortality during 2008–2019, to compare results of underlying vs. multiple cause of death analyses, and to describe the impact of the COVID-19 epidemic in 2020. We identified IPF (ICD-10 code J84.1) among the causes of death registered in 557,932 certificates in the Veneto region. We assessed time trends in annual age-standardized mortality rates by gender and age (40–74, 75–84, and ≥85 years). IPF was the underlying cause of 1310 deaths in the 2251 certificates mentioning IPF. For all age groups combined, the age-standardized mortality rate from IPF identified as the underlying cause of death was close to the European median (males and females: 3.1 and 1.3 per 100,000/year, respectively). During 2008–2019, mortality rates increased in men aged ≥85 years (annual percent change of 6.5%, 95% CI: 2.0, 11.2%), but not among women or for the younger age groups. A 72% excess of IPF-related deaths was registered in March–April 2020 (mortality ratio 1.72, 95% CI: 1.29, 2.24). IPF mortality was increasing among older men in northeastern Italy. The burden of IPF was heavier than assessed by routine statistics, since less than two out of three IPF-related deaths were directly attributed to this condition. COVID-19 was accompanied by a remarkable increase in IPF-related mortality.

PRS78 Burden of Idiopathic Pulmonary Fibrosis (IPF) on Quality of Life (QOL), Work Productivity and Healthcare Use

Despite recent advances, current IPF therapies fail to halt progression and have limited impact on QoL. Hospitalization due to IPF is common. We studied real-world burden of IPF on QoL (EQ-5D), hospitalization, work impairment and healthcare visits. A large cross-sectional survey was conducted in France, Germany, Japan and the USA; 244 physicians reported data on 1,249 patients with IPF, 739 of whom provided self-reported data. EQ-5D health utility values in IPF were worse than moderate-to-severe chronic obstructive pulmonary disease and similar to non-small cell lung cancer, based on comparative real-world data. Those on treatment (84.7%) consulted a healthcare professional more than those untreated (8.5 vs 5.2 visits/year); 33% and 10% made ≥10 visits/year, respectively, largely for routine check-ups and repeat prescriptions. Hospitalization was more common when disease was perceived as severe by physicians, but 5–17% of patients with mild functional impairment were still hospitalized in the last year. Work productivity was impaired by 28%, 45%, 40% and 34% in France, Germany, Japan and USA, respectively. Data show the marked burden of IPF on QoL, work productivity and healthcare resource use, highlighting the need for optimization of current management. ACKNOWLEDGMENTS : This study was funded by Galapagos NV (Mechelen, Belgium). Writing support from Hannah Mace MPharmacol, CMPP (Aspire Scientific Ltd, Bollington, UK) was funded by Galapagos NV.

Mortality Trends of Idiopathic Pulmonary Fibrosis in the United States From 2004 Through 2017

The burden of idiopathic pulmonary fibrosis (IPF)-related mortality in the United States in recent years is not well characterized. What are the trends in IPF-related mortality rates in the United States from 2004 through 2017? We used the Multiple Cause of Death Database available through the Centers for Disease Control and Prevention website, which contains data from all deceased US residents. IPF-related deaths were identified using International Classification of Diseases, 10th revision, codes. We examined annual trends in age-adjusted mortality rates stratified by age, sex, race, and state of residence. We also evaluated trends in place of death and underlying cause of death. From 2004 through 2017, the age-adjusted mortality decreased by 4.1%in men (from 75.5 deaths/1,000,000 in 2004 to 72.4 deaths/1,000,000 in 2017) and by 13.4%in women (from 46.3 deaths/1,000,000 in 2004 to 40.1 deaths/1,000,000 in 2017). This overall decrease was driven mainly by a decline in IPF-related mortality in patients younger than 85 years. The decreasing trend also was noted in all races except White men, in whom the rate remained stable. The most common cause of death was pulmonary fibrosis. The percentage of deaths occurring in the inpatient setting and nursing homes decreased, whereas the percentage of deaths occurring at home and hospice increased. From 2004 through 2017, the IPF age-adjusted mortality rates decreased. This may be explained partly by a decline in smoking in the United States, but further research is needed to evaluate other environmental and genetic contributors.

Burden of idiopathic pulmonary fibrosis on patients' emotional well being and quality of life: a literature review.

To present an overview of the impact of idiopathic pulmonary fibrosis (IPF) on patients' emotional well being and quality of life (QoL). IPF is an interstitial lung disease which causes irreversible, progressive lung scarring in a pathological pattern of usual interstitial pneumonia. The incidence of IPF is increasing at a global level, subjecting an increasing number of people to its high morbidity and risk of mortality. Diagnosis is based on a multidisciplinary team approach and the exclusion of other interstitial lung diseases. Two novel antifibrotic treatments, pirfenidone and nintedanib, were recently approved by regulatory agencies around the globe, thus providing many IPF patients with treatment options for the first time. Several other drugs have entered the investigational pipeline, including many in early-phase or late-phase clinical trials. Given the incurable and progressive nature of IPF, even with antifibrotic therapy, depression and anxiety are common among patients; these and burdensome symptoms of breathlessness, cough and fatigue are factors that impact patients' emotional well being and QoL. In addition to even more effective drugs, there is a need for psychosocial interventions and mental health support strategies focused on improving patients' QoL so they are better equipped to live with this devastating condition. The current article highlights the effects of IPF on patients' emotional well being and QoL and offers suggestions for strategies to help patients with IPF live as well as possible in their daily lives.

Clinical and economic burden of idiopathic pulmonary fibrosis in Quebec, Canada.

BackgroundIdiopathic pulmonary fibrosis (IPF), although rare, is a severe and costly disease.ObjectiveTo estimate the clinical and economic burden of IPF over multiple years before and after diagnosis using comprehensive administrative databases for the province of Quebec, Canada.MethodsSeveral administrative databases from Quebec, providing information on hospital care, community care, and pharmaceuticals, were linked over a 5-year period ending March 31, 2011, which was before approval of antifibrotic drugs in Canada. Prevalent and incident IPF cases were defined using International Classification Disease-10-CA codes and International Classification Disease-9-CM codes. We used a broad definition that excluded cases with subsequent diagnosis of other interstitial lung diseases and a narrow definition that required further diagnostic testing to confirm IPF diagnosis. Incident cases had an IPF code in a particular year without any IPF code in the 2 previous years. Health care resource utilization before and after the index diagnosis date was determined and costs calculated. Costs were expressed in 2016 Canadian dollars.ResultsOver 5-years, 10,579 (mean age: 76.4; 58% male) satisfied the broad definition of IPF and 8,683 (mean age: 74.5; 57% male) satisfied the narrow definition (82% of broad). Incidences of IPF overall were 25.8 and 21.7/100,000 population for broad and narrow definitions, respectively. Three-year survival was 40% and 37% in broad and narrow cohorts, respectively. For both cohorts, health care resource utilization and costs increased several years before diagnosis ($2,721 and $7,049/patient 5 years and 2 years prior to diagnosis using a broad definition, respectively) and remained elevated for multiple years post diagnosis ($12,978 and $8,267 at 2 and 3 years postdiagnosis).ConclusionHealth care resource utilization and costs of IPF increase many years prior to diagnosis. Incorporating multiyear annual costs before and after diagnosis results in a higher estimate of the economic burden of IPF than previous studies using a 1-year time frame.

Clinical and economic burden of idiopathic pulmonary fibrosis: a retrospective cohort study

BackgroundIdiopathic pulmonary fibrosis (IPF) is a devastating condition with a variable course. Not uncommonly, IPF patients are hospitalized for respiratory-related causes, including disease worsening. This study aimed to characterize the prevalence, and economic and health care burden of IPF.MethodsRetrospective insurance claims data collected yearly between January 1, 2009 and December 31, 2011, were used to determine prevalence and calculate all-cause and respiratory-related resource utilization and costs. Patients had at least one inpatient claim or two outpatient claims for IPF (ICD-9-CM code 516.3). Results for health care burden are reported for the 2011 cohort (similar findings in 2009–2010). Costs are reported in 2011 US dollars ($).ResultsPatients with IPF had a mean age of 69.8–71.3 years. Overall prevalence for IPF was 28.8, 28.1 and 19.8 per 100,000 insured persons in 2009, 2010 and 2011. In each year, prevalence increased with age. In 2011, 37.7 % of patients were hospitalized at least once for any cause; 19.5 % for respiratory-related reasons. Also in 2011, the mean number of all-cause outpatient visits and respiratory-related office visits was 18.5 and 5.7 per patient, respectively. All-cause health care costs in 2011 were $59,379 per patient; 36.6 % of costs ($21,732) were respiratory related.ConclusionsThe prevalence of IPF in this claims database increased with age, with a notable increase in those over 65 years. IPF is associated with a large economic and health care burden. Additional research is needed to determine how such burden might be reduced.

Asbestosis and environmental causes of usual interstitial pneumonia.

Recent epidemiologic investigations suggest that occupational and environmental exposures contribute to the overall burden of idiopathic pulmonary fibrosis (IPF). This article explores the epidemiologic and clinical challenges to establishing exposure associations, the current literature regarding exposure disease relationships and the diagnostic work-up of IPF and asbestosis patients. IPF patients demonstrate a histopathologic pattern of usual interstitial pneumonia. In the absence of a known cause or association, a usual interstitial pneumonia pattern leads to an IPF diagnosis, which is a progressive and often terminal fibrotic lung disease. It has long been recognized that asbestos exposure can cause pathologic and radiographic changes indistinguishable from IPF. Several epidemiologic studies, primarily case control in design, have found that a number of other exposures that can increase risk of developing IPF include cigarette smoke, wood dust, metal dust, sand/silica and agricultural exposures. Lung mineralogic analyses have provided additional support to causal associations. Genetic variation may explain differences in disease susceptibility among the population. An accumulating body of literature suggests that occupational and environmental exposure can contribute to the development of IPF. The impact of exposure on the pathogenesis and clinical course of disease requires further study.

The Increasing Secondary Care Burden of Idiopathic Pulmonary Fibrosis: Hospital Admission Trends in England From 1998 to 2010

The lack of mandatory clinical registries for idiopathic pulmonary fibrosis (IPF) has meant a reliance on routine clinical data to provide trends in disease incidence. Death certificate and primary care data suggest that the incidence of IPF has increased in the United Kingdom at a rate of approximately 5% per year, but due to possible concerns about the diagnostic validity of these clinical datasets, it is helpful also to analyze data from secondary care. We used national secondary care data to determine trends in hospital admissions for IPF clinical syndrome (IPF-CS) in England between 1998 and 2010. We obtained the annual number of hospital admissions for all National Health Service (NHS) hospital trusts in England for the International Classification of Diseases and Related Health Problems, 10th Revision codes J84.1 and J84.9 between 1998 and 2010. We calculated annual admission-rate ratios, adjusting for age and sex, using Poisson regression. We also investigated changes in age at admission and length of stay, and we estimated hospitalization costs. The number of hospital admissions from IPF-CS increased at an annual rate of approximately 5%, from 5,524 patients in 1998 to 9,525 patients in 2010, and was highest in men and the older population. Mean age at admission increased from 66 to 71 years, while length of stay decreased by 2.1 days during the same period. Hospital admissions for IPF-CS in England follow a similar trend to other data sources in the United Kingdom . This has resulted in escalating costs of inpatient care, which is a significant financial burden on health-care resources.