Unravelling the health and economic burden of interstitial lung diseases in adults in Australia.

BACKGROUND: Interstitial lung diseases (ILD) are a heterogenous group of over 200 disorders affecting the pulmonary interstitium. Although there have been advances in knowledge on ILDs in Australia, the characterisation of the health and economic burden of disease remained largely undetermined until recently. OBJECTIVE: The main objective of this review is to provide a synopsis of health and economic burden of ILDs in Australia, based on recently completed research. DISCUSSION: Recent research has demonstrated that idiopathic pulmonary fibrosis (IPF) is the most frequent ILD in Australia. Incidence and prevalence of IPF have demonstrated an increasing trend over the past decades. Mortality has also increased over the past decades, but has shown a slight decreasing trend recently, since the introduction of antifibrotic medication. Health-related quality of life is poor in patients with IPF, and care is estimated to cost approximately AU$299 million per year in Australia. Early diagnosis and referral to tertiary care is crucial for favourable outcomes, and general practitioners are considerably important to this as the first interface to identify patients at risk and detect early symptoms of ILDs.

Epidemiology, healthcare utilization, and related costs among patients with IPF: results from a German claims database analysis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive form of fibrosing interstitial pneumonia with poor survival. This study provides insight into the epidemiology, cost, and disease course of IPF in Germany. METHODS: A cohort of incident patients with IPF (n = 1737) was identified from German claims data (2014-2019). Incidence and prevalence rates were calculated and adjusted for age differences compared with the overall German population. All-cause and IPF-related healthcare resource utilization as well as associated costs were evaluated per observed person-year (PY) following the initial IPF diagnosis. Finally, Kaplan-Meier analyses were performed to assess time from initial diagnosis to disease deterioration (using three proxy measures: non-elective hospitalization, IPF-related hospitalization, long-term oxygen therapy [LTOT]); antifibrotic therapy initiation; and all-cause death. RESULTS: The cumulative incidence of IPF was estimated at 10.7 per 100,000 individuals in 2016, 10.9 in 2017, 10.5 in 2018, and 9.6 in 2019. The point prevalence rates per 100,000 individuals for the respective years were 21.7, 23.5, 24.1, and 24.1. On average, ≥ 14 physician visits and nearly two hospitalizations per PY were observed after the initial IPF diagnosis. Of total all-cause direct costs (€15,721/PY), 55.7% (€8754/PY) were due to hospitalizations and 29.1% (€4572/PY) were due to medication. Medication accounted for 49.4% (€1470/PY) and hospitalizations for 34.8% (€1034/PY) of total IPF-related direct costs (€2973/PY). Within 2 years of the initial IPF diagnosis (23.6 months), 25% of patients died. Within 5 years of diagnosis, 53.1% of patients had initiated LTOT; only 11.6% were treated with antifibrotic agents. The median time from the initial diagnosis to the first non-elective hospitalization was 5.5 months. CONCLUSION: The incidence and prevalence of IPF in Germany are at the higher end of the range reported in the literature. The main driver for all-cause cost was hospitalization. IPF-related costs were mainly driven by medication, with antifibrotic agents accounting for around one-third of the total medication costs even if not frequently prescribed. Most patients with IPF do not receive pharmacological treatment, highlighting the existing unmet medical need for effective and well-tolerated therapies.

Cost-effectiveness of the anti-fibrotics for the treatment of idiopathic pulmonary fibrosis in the United States.

BACKGROUND: The anti-fibrotic medications nintedanib and pirfenidone were approved in the United States for use in patients with idiopathic pulmonary fibrosis several years ago. While there is a growing body of evidence surrounding their clinical effectiveness, these medications are quite expensive and no prior cost-effectiveness analysis has been performed in the United States. METHODS: A previously published Markov model performed in the United Kingdom was replicated using United States data to project the lifetime costs and health benefits of treating idiopathic pulmonary fibrosis with: (1) symptom management; (2) pirfenidone; or (3) nintedanib. For the cost-effectiveness analysis, strategies were ranked by increasing costs and then checked for dominating treatment strategies. Then an incremental cost-effectiveness ratio was calculated for the dominant therapy. RESULTS: The anti-fibrotic medications were found to cost more than $110,000 per year compared to $12,291 annually for symptom management. While pirfenidone was slightly more expensive than nintedanib and provided the same amount of benefit, neither medication was found to be cost-effective in this U.S.-based analysis, with an average cost of $1.6 million to gain one additional quality-adjusted life year over symptom management. CONCLUSIONS: Though the anti-fibrotics remain the only effective treatment option for patients with idiopathic pulmonary fibrosis and the data surrounding their clinical effectiveness continues to grow, they are not considered cost-effective treatment strategies in the United States due to their high price.

Neighborhood-Level Disadvantage Impacts on Patients with Fibrotic Interstitial Lung Disease.

Rationale: Fibrotic interstitial lung disease (fILD) is a group of pathologic entities characterized by scarring of the lungs and high morbidity and mortality. Research investigating how socioeconomic and residential factors impact outcomes in patients with fILD is lacking. Objectives: To determine the association between neighborhood-level disadvantage and presentation severity, disease progression, lung transplantation, and mortality in patients with fILD from the United States and Canada. Methods: We performed a multicenter, international, prospective cohort study of 4,729 patients with fILD from one U.S. and eight Canadian ILD registry sites. Neighborhood-level disadvantage was measured by the area deprivation index in the United States and the Canadian Index of Multiple Deprivation in Canada. Measurements and Main Results: In the U.S. but not in the Canadian cohort, patients with fILD living in neighborhoods with the greatest disadvantage (top quartile) experience the highest risk of mortality (hazard ratio = 1.51, P = 0.002), and in subgroups of patients with idiopathic pulmonary fibrosis, the top quartile of disadvantage experienced the lowest odds of lung transplantation (odds ratio = 0.46, P = 0.04). Greater disadvantage was associated with reduced baseline DLCO in both cohorts, but it was not associated with baseline FVC or FVC or DLCO decline in either cohort. Conclusions: Patients with fILD who live in areas with greater neighborhood-level disadvantage in the United States experience higher mortality, and patients with idiopathic pulmonary fibrosis experience lower odds of lung transplantation. These disparities are not seen in Canadian patients, which may indicate differences in access to care between the United States and Canada.

Pirfenidone vs. nintedanib in patients with idiopathic pulmonary fibrosis: a retrospective cohort study.

BACKGROUND: Two antifibrotic drugs, pirfenidone and nintedanib, are licensed for the treatment of patients with idiopathic pulmonary fibrosis (IPF). However, there is neither evidence from prospective data nor a guideline recommendation, which drug should be preferred over the other. This study aimed to compare pirfenidone and nintedanib-treated patients regarding all-cause mortality, all-cause and respiratory-related hospitalizations, and overall as well as respiratory-related health care costs borne by the Statutory Health Insurance (SHI). METHODS: A retrospective cohort study with SHI data was performed, including IPF patients treated either with pirfenidone or nintedanib. Stabilized inverse probability of treatment weighting (IPTW) based on propensity scores was applied to adjust for observed covariates. Weighted Cox models were estimated to analyze mortality and hospitalization. Weighted cost differences with bootstrapped 95% confidence intervals (CI) were applied for cost analysis. RESULTS: We compared 840 patients treated with pirfenidone and 713 patients treated with nintedanib. Both groups were similar regarding two-year all-cause mortality (HR: 0.90 95% CI: 0.76; 1.07), one-year all cause (HR: 1.09, 95% CI: 0.95; 1.25) and respiratory-related hospitalization (HR: 0.89, 95% CI: 0.72; 1.08). No significant differences were observed regarding total (€- 807, 95% CI: €- 2977; €1220) and respiratory-related (€- 1282, 95% CI: €- 3423; €534) costs. CONCLUSION: Our analyses suggest that the patient-related outcomes mortality, hospitalization, and costs do not differ between the two currently available antifibrotic drugs pirfenidone and nintedanib. Hence, the decision on treatment with pirfenidone versus treatment with nintedanib ought to be made case-by-case taking clinical characteristics, comorbidities, comedications, individual risk of side effects, and patients' preferences into account.

Clusters of comorbidities in idiopathic pulmonary fibrosis.

INTRODUCTION: Comorbidities are common in patients with idiopathic pulmonary fibrosis (IPF) and negatively impact health-related quality of life, health-care costs and mortality. Retrospective studies have focused on individual comorbidities, but clusters of multiple comorbidities have rarely been analysed. This study aimed to comprehensively and prospectively assess comorbidities in a multicentre, real-world cohort of patients with IPF, including prespecified conditions of special interest and to analyse clusters of comorbidities and examine characteristics, disease course and mortality of the clusters. METHODS: Several measurements, questionnaires, medications and medical history were combined to assess comorbidities. Using self-organizing maps, clusters of comorbidities were identified and phenotypes characterized. Disease course was assessed using mixed effects models and mortality using Cox regression. RESULTS: One-hundred and fifty IPF patients were included prospectively. All except one patient suffered from at least one comorbidity and multimorbidity was common. Arterial hypertension, gastro-oesophageal reflux disease, hypercholesterolemia, emphysema and obstructive sleep apnea were most prevalent. Four comorbidity clusters were identified. Each cluster had distinct comorbidity profiles, patient characteristics, symptom burden and disease severity. Patients with fewer comorbidities had better exercise capacity and less dyspnea at baseline, but a trend towards faster deterioration was observed. Mortality analyses showed no significant differences between clusters. CONCLUSIONS: Multimorbidity is prevalent in patients with IPF. Four specific clusters of comorbidities may represent phenotypes in IPF. A trend towards faster decline in exercise capacity and dyspnea was observed in patients with fewer comorbidities. Increased knowledge of comorbidities facilitates prevention and treatment of comorbidities in patients with IPF.

Low income and outcome in idiopathic pulmonary fibrosis: An association to uncover.

BACKGROUND: Low income, a known prognostic indicator of various chronic respiratory diseases, has not been properly studied in idiopathic pulmonary fibrosis (IPF). We hypothesize that a low income has an adverse prognostic impact on IPF. METHODS: Patients were selected from the French national prospective cohort COFI. Patients' income was assessed through the median city-level income provided by the French National Institute of Statistics and Economic Studies according to their residential address. Patients were classified in two groups as "low income" vs. "higher income" depending on whether their annual income was estimated to be < or ≥18 170 €/year (the first quartile of the income distribution in the study population). The survival and progression-free survival (PFS) of the groups were compared by a log-rank test and a Cox model in multivariate analysis. RESULTS: 200 patients were included. The average follow-up was 33.8 ± 22.7 months. Patients in the low income group were significantly more likely to be of non-European origin (p < 0.006), and to have at least one occupational exposure (p < 0.0001), and they tended to have a higher cumulative exposure to fine particles PM2.5 (p = 0.057). After adjusting for age, gender, forced vital capacity at inclusion, geographical origin, and occupational exposure having a low-income level was a factor associated with a worse PFS (HR: 1.81; CI95%: 1.24-2.62, p = 0.001) and overall survival (HR: 1.49; CI95%: 1.0006-2.23, p = 0.049). CONCLUSIONS: Low income appears to be a prognostic factor in IPF. IPF patients with low incomes may also be exposed more frequently to occupational exposures.

Healthcare use and costs among Medicare enrollees on pirfenidone versus nintedanib for idiopathic pulmonary fibrosis.

Aim: Compare healthcare utilization and costs between Medicare beneficiaries with idiopathic pulmonary fibrosis (IPF) receiving pirfenidone or nintedanib. Methods: Retrospective cohort study of Medicare beneficiaries (100% Research Identifiable Files) with IPF who initiated pirfenidone or nintedanib between 15 October 2014 and 31 December 2015. Inverse probability of treatment weighting using propensity scores adjusted for baseline covariates. Outcomes: hospitalization and monthly costs. Results: Hazard and incidence rate ratios (95% CI) for all-cause (0.79 [0.68-0.91]; 0.69 [0.59-0.82]) and respiratory-related (0.80 [0.65-0.97]; 0.71 [0.57-0.90]) hospitalizations favored pirfenidone versus nintedanib. Monthly inpatient costs were lower for pirfenidone versus nintedanib patients; outpatient and pharmacy costs were similar. Conclusion: In patients with IPF, pirfenidone compared with nintedanib has a moderate but significant protective effect on hospitalization, corresponding to lower inpatient costs.

Idiopathic Pulmonary Fibrosis: Utilization of Health Services and Out-Of-Pocket Health Expenditures in Greece.

OBJECTIVES: Idiopathic pulmonary fibrosis (IPF) is a severe progressive disease with poor prognosis. Patients show an increased healthcare utilization pattern consisting of regular physician consultations and monitoring tests. The aim of the present study was to estimate healthcare resource utilization and out-of-pocket expenditures for IPF patients in Greece. METHODS: An IPF-specific questionnaire was constructed, which focused on retrospective healthcare resource utilization and out-of-pocket expenditures within a 6-month period. Upon informed consent, the questionnaires were completed anonymously. RESULTS: The study sample included 123 IPF patients. Within a 6-month period, 85.4% of the patients visited their treating physician (mean, 2.35; standard deviation [SD], 1.400), 29.4% visited other physicians for IPF-related reasons (mean, 1.90; SD, 1.595), 42.9% visited a healthcare setting, and 10.6% required hospitalization (mean duration, 2.86 days; SD, 3.338). Spirometry (98.1%) and blood tests (88.5%) were the most common tests for IPF monitoring. Almost half of the respondents (48.6%) stated they had private expenditures for IPF medication, physician visits, hospital services, medical tests, or other IPF-related reasons. CONCLUSIONS: IPF management requires regular contact with healthcare services and uptake of specific medical tests. Considering the high proportion of patients bearing out-of-pocket costs, along with the decline of incomes in Greece, improvement of the disease management is important, and action should be taken to lower the financial burden imposed on patients.

Treatment patterns, healthcare resource utilization, and costs among patients with idiopathic pulmonary fibrosis treated with antifibrotic medications in US-based commercial and Medicare Supplemental claims databases: a retrospective cohort study.

BACKGROUND: Pirfenidone and nintedanib are antifibrotic therapies which slow disease progression in idiopathic pulmonary fibrosis (IPF), an irreversible, progressive lung disease with poor prognosis. We compared adherence, persistence, and healthcare costs between patients initiating one of the two therapies. METHODS: We used the IBM Watson Health Commercial and Medicare Supplemental claims databases to select patients with IPF with ≥1 pharmacy claim for pirfenidone or nintedanib between 10/1/2014 and 6/30/2018. Adherence (proportion of days covered ≥0.80) and persistence (time to a gap of ≥60 days without medication or switch to the other antifibrotic medication) based on the days' supply and service date fields on claims were measured over a variable-length follow-up period. Healthcare costs, all-cause and respiratory-related, were measured over the persistent period and a fixed 12-month follow-up period. Inverse probability of treatment weights were applied to models comparing adherence, persistence, and costs between the two cohorts. RESULTS: Overall, 799 pirfenidone patients and 656 nintedanib patients were identified. Similar proportions of patients were adherent in both cohorts (pirfenidone = 49% vs. nintedanib = 51%) and there was no significant difference in the odds of being adherent after weighting (odds ratio = 1.1, p = 0.513). The proportions of patients who discontinued/switched were also similar (pirfenidone = 41% vs. nintedanib 43%); however, in a weighted model, the hazards of discontinuation/switching was lower for the pirfenidone cohort (hazard ratio = 0.8, p = 0.032). While patients were persistent on therapy, weighted all-cause healthcare costs were comparable (pirfenidone = $11,272 vs. nintedanib = $11,987 per-patient per-month; p = 0.115), but weighted respiratory-related costs were significantly lower for the pirfenidone cohort ($9015 vs. $10,167 per-patient per-month, p < 0.001). Weighted annual total all-cause and respiratory-related healthcare costs were comparable between cohorts over the fixed 12-month follow-up period, but the pirfenidone cohort had significantly lower weighted annual mean antifibrotic drug costs than the nintedanib cohort ($68,850 vs. $77,033, p = 0.007). CONCLUSIONS: Pirfenidone use was associated with longer time to discontinuation/switch, lower antifibrotic drug costs, and lower respiratory-related total costs compared to nintedanib use.

The Burden of Progressive Fibrosing Interstitial Lung Disease: A DELPHI Approach.

INTRODUCTION: The term progressive fibrosing interstitial lung disease (ILD) describes patients with fibrotic ILDs who, irrespective of the aetiology of the disease, show a progressive course of their disease despite current available (and non-licensed) treatment. Besides in idiopathic pulmonary fibrosis, little is known about management and the burden of patients with fibrotic ILD, particularly those with a progressive behaviour. METHODS: Using the Delphi method, 40 European experts in ILD management delivered information on management of (progressive) fibrosing ILD and on the impact of the disease on patients' quality of life (QoL) and healthcare resource utilisation (HCRU). Annual costs were calculated for progressive and non-/slow-progressive fibrosing ILD for diagnosis, follow-up management, exacerbation management, and end-of-life care based on the survey data. RESULTS: Physicians reported that progression in fibrosing ILD worsens QoL in both patients and their caregivers. Progression of fibrosing ILD was associated with a greater use of HCRU for follow-up visits and maintenance treatment compared with the non-/slow progression. The number of patients who suffered at least one acute exacerbation was reported to be more than three times higher in progressive fibrosing ILD patients than in patients with non-/slow-progressive fibrosing ILD. On average, annual estimated costs of progressive fibrosing ILD per patient were 1.8 times higher than those of the non-/slow-progressive form of the disease. CONCLUSIONS: Progression in fibrosing ILD causes a significant impact on QoL and HCRU and costs. These survey data underline the need for safe and effective therapies to slow the disease progression.

Healthcare Resources Utilization and Costs of Patients with Non-IPF Progressive Fibrosing Interstitial Lung Disease Based on Insurance Claims in the USA.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is the classic progressive fibrosing interstitial lung disease (ILD), but some patients with ILDs other than IPF also develop a progressive fibrosing phenotype (PF-ILD). Information on use and cost of healthcare resources in patients with PF-ILD is limited. METHODS: We used USA-based medical insurance claims (2014-2016) to assess use and cost of healthcare resources in PF-ILD. Patients with at least two ILD claims and at least one pulmonologist visit were considered to have ILD. Pulmonologist visit frequency was used as a proxy to identify PF-ILD (at least four visits in 2016, or at least three more visits in 2016 vs. 2014). RESULTS: Of 2517 patients with non-IPF ILD, 15% (n = 373) had PF-ILD. Mean annual medical costs associated with ILD claims were $35,364 in patients with non-IPF PF-ILD versus $20,211 in the non-IPF ILD population. In 2016, patients with non-IPF PF-ILD made more hospital ILD claims than patients with non-IPF ILD (10.5 vs. 4.7). CONCLUSIONS: These findings suggest higher disease severity and overall healthcare use for patients with a non-IPF ILD manifesting a progressive fibrosing phenotype (non-IPF PF-ILD).

Interstitial lung disease (ILD) is a group of similar lung conditions with lung fibrosis, scarring, or inflammation of the lung tissue. Some patients with ILD also have worsening lung fibrosis, referred to as "progressive fibrosis" (PF-ILD). The most common type of PF-ILD is idiopathic pulmonary fibrosis (IPF), which has no known cause. Although much is known about IPF, there is limited information available on how often patients with ILDs other than IPF (non-IPF ILD) use healthcare, or the costs associated with the disease. This study used US medical insurance claims to gain further insights. The study examined data from over 2500 patients with non-IPF ILD, of which 15% had PF-ILD. Patients defined as having PF-ILD had higher yearly medical costs and used healthcare services more often than other patients with ILD. This study highlights the economic burden of non-IPF ILD with progressive fibrosis (non-IPF PF-ILD).

Health Care Costs at the End of Life for Patients with Idiopathic Pulmonary Fibrosis. Evaluation of a Pilot Multidisciplinary Collaborative Interstitial Lung Disease Clinic.

Rationale: Even though idiopathic pulmonary fibrosis (IPF) is a disease with high morbidity and mortality and no cure, palliative care is rarely implemented, leading to high symptom burden and unmet care needs. In 2012, we implemented a multidisciplinary collaborative (MDC) care model linking clinic and community multidisciplinary teams to provide an early integrated palliative approach, focusing on early symptom management and advance care planning.Objectives: To evaluate the differences in resource use and associated costs of end-of-life care between patients with IPF who received early integrated palliative care and patients with IPF who received conventional treatment.Methods: Using administrative health data, we identified all patients in the Province of Alberta, Canada, who presented to a hospital with an IPF diagnosis between January 1, 2012, and December 31, 2018, and died within this time frame. We compared three groups of patients: those who received MDC care (our clinic patients), specialist care (SC; respirologist), or non-specialist care (NSC; no contact with a respiratory clinic). The primary outcomes were healthcare resource use and costs in the year before death.Results: Of 2,768 patients across the three study groups, in the last year of life, MDC patients were more than three times as likely as SC patients to have received antifibrotic therapies (odds ratio [OR], 3.0; 95% confidence interval [CI], 1.8-5.2), almost twice as likely to have received pulmonary rehabilitation (OR, 1.9; 95% CI, 1.1-3.4), and 36% more likely to have received opiates (OR, 1.4; 95% CI, 0.8-2.3). The median total healthcare costs in the last 3 months of life were approximately C$7,700 lower for MDC patients than for those receiving SC, driven primarily by fewer hospitalizations and emergency department visits. MDC patients were also less likely to die in the hospital (44.9% MDC vs. 64.9% SC vs. 66.8% NSC; P < 0.001) and had the highest rates of no hospitalization in the last year of life.Conclusions: An integrated palliative approach in IPF is associated with improvements in the quality of end-of-life care and reduction in costs. Transformation of care models is required to deliver palliative care for patients with IPF. MDC teams within such models can address the high burden of unmet needs for symptom management, advance care planning, and community support in this complex population.

Hospital-Based Resource Use and Costs Among Patients With Idiopathic Pulmonary Fibrosis Enrolled in the Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a rare and serious condition that is associated with high health-care resource use. The goal of this study was to estimate hospital-related resource use and costs by using a national, prospective registry of patients who were diagnosed with IPF or who had their diagnosis confirmed at the enrolling center in the past 6 months in the United States. METHODS: Participants enrolled between June 5, 2014, and April 12, 2016, in the ongoing Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry were included (N = 300). Time to first hospitalization was analyzed by using Kaplan-Meier methods. Annualized costs were estimated for hospitalizations, ICU admissions, and ED visits. RESULTS: At enrollment, most participants were male (75%), white (95%), commercially insured (64%), smokers (68%), had an FVC between 50% and 80% predicted (66%), and received antifibrotic drugs (55%). During the first 12 months of follow-up, participants averaged 0.11 ED visit, 0.42 hospitalization, 0.08 ICU admission, 2.18 hospital days, and 0.45 ICU day. Probability of hospitalization was 18% and 30% at 6 and 12 months, respectively, and was highest for those with FVC < 50% predicted/diffusing lung capacity for carbon monoxide < 30% predicted. Mean annual costs (95% CI) for ICU admission and inpatient care were $10,098 ($4,732-$16,662) and $13,975 ($8,482-$20,918), respectively, per patient. CONCLUSIONS: IPF is associated with a substantial economic burden incurred by patients requiring hospital care. Future research in IPF should focus on improving clinical outcomes while reducing cost of care in hospitals. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT01915511; URL: www.clinicaltrials.gov.

Characterizing Health Outcomes in Idiopathic Pulmonary Fibrosis using US Health Claims Data.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a life-threatening interstitial lung disease (ILD). Characterizing health outcomes of IPF patients is challenging due to disease rarity. OBJECTIVE: This study aimed to identify the burden of disease in patients newly diagnosed with IPF. METHODS: Patients with ≥1 claim with an IPF diagnosis were identified from a United States healthcare insurer's database (2000-2013). Patients with other known causes of ILD or aged <40 years were excluded. Subgroups were compared based on the 2011 change in International Classification of Diseases, 9th Revision (ICD-9) definition of IPF and occurrence of IPF testing. The prevalence and incidence of preselected health conditions of clinical interest were estimated. RESULTS: Median age of newly diagnosed patients (n = 7,298) was 62 years (54.0% male). Restricting to patients with IPF diagnostic testing did not substantially affect cohort characteristics, nor did ICD-9 IPF coding change. Mean follow-up was 1.7 years; 16.8% of patients died; and a substantial proportion of patients were censored due to end of health plan enrollment (50.7%) and other causes of ILD (19.6%). The incidence of pulmonary hypertension, lung cancer, and claims-based algorithm proxy for acute respiratory worsening of unknown cause was 22.5, 17.6, and 12.6 per 1,000 person-years, respectively. CONCLUSIONS: Patients with IPF had a high disease burden with a variety of health outcomes observed, including a high rate of mortality. Database censoring due to changes in enrollment or other ILD diagnoses limited follow-up. Altering cohort entry definitions, including IPF testing or ICD-9 IPF coding change, had little impact on cohort baseline characteristics.

Improving outcomes and managing costs in idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic pulmonary disease that is complicated by diagnostic challenges, multiple comorbidities, and a poor prognosis. Although considered a relatively rare disease, healthcare costs are substantial and disproportionate to the incidence and prevalence of the disease. The comorbidities associated with IPF not only complicate treatment strategies but also increase the burden for patients via higher costs and undesirable health outcomes. Historically, pharmacologic treatment options for IPF have been limited and are often associated with low efficacy. Two drugs approved for IPF, nintedanib and pirfenidone, offer promise for improving health outcomes and survival during the course of the disease. Considerations of cost and adverse events are important when planning treatment options. Optimizing care through patient-centered care management programs can improve outcomes and health-related quality of life for patients. Such programs emphasize communication between healthcare professionals and patients in order to educate patients on their condition, so they can make informed healthcare decisions. Disease registries can be important tools for optimizing data collection and analysis for a disease with limited incidence and prevalence.

Costs of Workplace Productivity Loss in Patients With Fibrotic Interstitial Lung Disease.

BACKGROUND: Fibrotic interstitial lung diseases (ILDs) are highly morbid chronic disorders that frequently occur in working age individuals. The goal of this study was to determine workplace productivity loss, its determinants, and its estimated costs in patients with fibrotic ILD. METHODS: Patients with idiopathic pulmonary fibrosis, chronic hypersensitivity pneumonitis, idiopathic nonspecific interstitial pneumonia, or unclassifiable ILD were identified from the six-center Canadian Registry for Pulmonary Fibrosis (CARE-PF). The Work Productivity and Activity Impairment questionnaire was used to determine health-related productivity loss. Independent predictors of low workplace productivity were identified by using multivariate regression. Patient data were compared with Canadian population census data. The average productivity loss (hours per week) and the individual's hourly wage were used to estimate the costs of productivity loss. RESULTS: Of 650 eligible patients, 148 (23%) were employed. Productivity loss was reported by 55% of employed patients with an average productivity loss of 7.8 ± 0.9 h per week (2.3 ± 0.6 h per week related to absenteeism and 5.5 ± 0.6 h per week related to presenteeism). Employment among patients with ILD aged 25 to 54 years was 23% lower than the age- and sex-matched general Canadian population (60% vs 83%; P < .001). Employment among patients with ILD aged ≥ 55 years was 18% lower than in the age- and sex-matched population (20% vs 38%; P < .001). Dyspnea and cough were independent predictors of workplace productivity loss. Estimated annual costs of productivity loss were 11,610 Canadian dollars per employee with ILD. CONCLUSIONS: Workplace productivity loss is common in fibrotic ILD, strongly correlated with symptom severity, and associated with significant cost.

The Burden of Illness of Idiopathic Pulmonary Fibrosis: A Comprehensive Evidence Review.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a debilitating condition with significant morbidity and poor survival. Since 2010, there has been increased activity in the development of treatments that aim to delay progression of the disease. OBJECTIVE: Our study involves a comprehensive review of the literature for evidence on health-related quality of life (HRQoL), healthcare resource use (HCRU) and costs, and an assessment of the burden of illness of the condition. METHODS: We carried out a systematic literature review (SLR) to identify economic evaluations and HRQoL studies. We searched EMBASE, MEDLINE and MEDLINE In Process for relevant studies from database origins to April 2017. Alongside the presentation of the study characteristics and the available evidence, we carried out a qualitative comparison using reference population estimates for HRQoL and national health expenditure for costs. RESULTS: Our search identified a total of 3241 records. After removing duplicates and not relevant articles, we analysed 124 publications referring to 88 studies published between 2000 and 2017. Sixty studies were HRQoL and 28 were studies on costs or HCRU. We observed an exponential growth of publications in the last 3-5 years, with the majority of the studies conducted in Europe and North America. Among the HRQoL studies, and despite regional differences, there was some agreement between estimates on the absolute and relative level of HRQoL for patients with IPF compared with the general population. Regarding costs, after adjustments for the cost years and currency, the suggested annual per capita cost of patients with IPF in North America was estimated around US$20,000, 2.5-3.5 times higher than the national healthcare expenditure. Additionally, studies that analysed patients with IPF alongside a matched control cohort suggested a significant increase in resource use and cost. CONCLUSION: The reviewed evidence indicates that IPF has considerable impact on HRQoL, relative to the general population levels. Furthermore, in studies of cost and resource use, most estimates of the burden were consistent in suggesting an excess cost for patients with IPF compared with a control cohort or the national health expenditure. This confirms IPF as a growing threat for public health worldwide, with considerable impact to the patients and healthcare providers.

Health economic evaluation in idiopathic pulmonary fibrosis in France.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause. To date, there is no specific cure for IPF, and only two treatments (pirfenidone and nintedanib) have marketing authorizations and recommendations in international and French guidelines. OBJECTIVES: A cost-utility analysis (CUA) has been conducted to evaluate the efficiency of nintedanib, in comparison to all available alternatives, in a French setting using the official methodological guidelines. METHODS: A previously developed lifetime Markov model was adapted to the French setting by simulating the progression of IPF patients in terms of lung function decline, incidence of acute exacerbations, and death. Considering the effect of IPF on patients' quality-of-life, a CUA integrating quality adjusted life years (QALY) was chosen as the primary outcome measure in the main analysis. One-way, probabilistic, and scenario sensitivity analyses were performed to evaluate the robustness of the model. RESULTS: Treatment with nintedanib resulted in an estimated total cost of €76,414 (vs €82,665 for pirfenidone). In comparison with all other available options, nintedanib was predicted to provide the most QALY gained (3.34 vs 3.29). This analysis suggests that nintedanib has a 59.0% chance of being more effective than pirfenidone and s 77.3% chance of being cheaper than pirfenidone. Sensitivity analyses showed the results of the CUA to be robust. CONCLUSIONS: In conclusion, this CUA has found that nintedanib appears to be a more cost-effective therapeutic option than pirfenidone in a French setting, due to fewer acute exacerbations and a better tolerability profile.