Care In Idiopathic Pulmonary Fibrosis Research Articles

64Cu]Cu-PEG-FUD peptide for noninvasive and sensitive detection of murine pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease resulting in irreversible scarring within the lungs. However, the lack of biomarkers that enable real-time assessment of disease activity remains a challenge in providing efficient clinical decision-making and optimal patient care in IPF. Fibronectin (FN) is highly expressed in fibroblastic foci of the IPF lung where active extracellular matrix (ECM) deposition occurs. Functional upstream domain (FUD) tightly binds the N-terminal 70-kilodalton domain of FN that is crucial for FN assembly. In this study, we first demonstrate the capacity of PEGylated FUD (PEG-FUD) to target FN deposition in human IPF tissue ex vivo. We subsequently radiolabeled PEG-FUD with 64Cu and monitored its spatiotemporal biodistribution via μPET/CT imaging in mice using the bleomycin-induced model of pulmonary injury and fibrosis. We demonstrated [64Cu]Cu-PEG-FUD uptake 3 and 11 days following bleomycin treatment, suggesting that radiolabeled PEG-FUD holds promise as an imaging probe in aiding the assessment of fibrotic lung disease activity.

Meaningful Endpoints for Idiopathic Pulmonary Fibrosis (IPF) Clinical Trials: Emphasis on 'Feels, Functions, Survives'. Report of a Collaborative Discussion in a Symposium with Direct Engagement from Representatives of Patients, Investigators, the National Institutes of Health, a Patient Advocacy Organization, and a Regulatory Agency.

Background: Idiopathic pulmonary fibrosis (IPF) carries significant mortality and unpredictable progression, with limited therapeutic options. Designing trials with patient-meaningful endpoints, enhancing the reliability and interpretability of results, and streamlining the regulatory approval process are of critical importance to advancing clinical care in IPF. Methods: A landmark in-person symposium in June 2023 assembled 43 participants from the US and internationally, including patients with IPF, investigators, and regulatory representatives, to discuss the immediate future of IPF clinical trial endpoints. Patient advocates were central to discussions, which evaluated endpoints according to regulatory standards and the FDA's 'feels, functions, survives' criteria. Results: Three themes emerged: 1) consensus on endpoints mirroring the lived experiences of patients with IPF; 2) consideration of replacing forced vital capacity (FVC) as the primary endpoint, potentially by composite endpoints that include 'feels, functions, survives' measures or FVC as components; 3) support for simplified, user-friendly patient-reported outcomes (PROs) as either components of primary composite endpoints or key secondary endpoints, supplemented by functional tests as secondary endpoints and novel biomarkers as supportive measures (FDA Guidance for Industry (Multiple Endpoints in Clinical Trials) available at: https://www.fda.gov/media/162416/download). Conclusions: This report, detailing the proceedings of this pivotal symposium, suggests a potential turning point in designing future IPF clinical trials more attuned to outcomes meaningful to patients, and documents the collective agreement across multidisciplinary stakeholders on the importance of anchoring IPF trial endpoints on real patient experiences-namely, how they feel, function, and survive. There is considerable optimism that clinical care in IPF will progress through trials focused on patient-centric insights, ultimately guiding transformative treatment strategies to enhance patients' quality of life and survival.

Dyspnea Associates With a Widely Impaired Quality of Life in Idiopathic Pulmonary Fibrosis Patients: A Longitudinal Study Using 15D.

Background: Research on health-related quality of life (HRQoL) is crucial for developing comprehensive palliative care in idiopathic pulmonary fibrosis (IPF). Objectives: To study IPF patients' HRQoL compared with general population and its association with dyspnea in a longitudinal follow-up. Design: Assessment of IPF patients' HRQoL by a generic tool. Comparison of baseline data with the general population and a 30-month follow-up with 6 months intervals. Setting/Subjects: In total, 246 IPF patients were recruited from the Finnish nationwide real-life study, FinnishIPF. Measurements: Modified Medical Research Council (MMRC) dyspnea scale for dyspnea and the generic HRQoL tool 15D for the total and dimensional HRQoL were used. Results: At baseline, the mean 15D total score was lower (0.786, standard deviation [SD] 0.116) in IPF patients than in the general population (0.871, SD 0.043) (p < 0.001) and among the IPF patients with MMRC ≥2 compared with those with MMRC <2 (p < 0.001). In patients with MMRC ≥2, significant impairment compared with general population existed in 11 dimensions of HRQoL, such as breathing, usual activities, and sexual activity, whereas this was true in only 4 dimensions in MMRC <2 category. Mental function was not impaired in either group. During the follow-up, 15D total score decreased in both MMRC categories (p < 0.001) but stayed constantly worse in the MMRC ≥2 group. Seven and two dimensions of HRQoL significantly declined in the categories of MMRC <2 and MMRC ≥2, respectively. Conclusions: Patients with IPF, especially if dyspnea limits everyday life, suffer from widely impaired HRQoL, although self-assessed mental capability is preserved. Integrated palliative care is supported to face the multiple needs of IPF patients.

Therapeutic decisions in a cohort of patients with idiopathic pulmonary fibrosis: a multicenter, prospective survey from Poland.

Background:Pirfenidone and nintedanib are considered as the standard of care in idiopathic pulmonary fibrosis (IPF), but there is no consensus as to which of these two agents should be regarded as first-line treatment.Objective:To provide real-world data on therapeutic decisions of pulmonary specialists, particularly the choice of the antifibrotic drug in patients with IPF.Methods:This was a multicenter, prospective survey collecting clinical data of patients with IPF considered as candidates for antifibrotic treatment between September 2019 and December 2020. Clinical characteristics and information on the therapeutic approach were retrieved. Statistical evaluation included multiple logistic regression analysis with stepwise model selection.Results:Data on 188 patients [74.5% male, median age 73 (interquartile range, 68–78) years] considered for antifibrotic therapy were collected. Treatment was initiated in 138 patients, while 50 patients did not receive an antifibrotic, mainly due to the lack of consent for treatment and IPF severity. Seventy-two patients received pirfenidone and 66 received nintedanib. Dosing protocol (p < 0.01) and patient preference (p = 0.049) were more frequently associated with the choice of nintedanib, while comorbidity profile (p = 0.0003) and concomitant medication use (p = 0.03) were more frequently associated with the choice of pirfenidone. Age (p = 0.002), lung transfer factor for carbon monoxide (TLCO) (p = 0.001), and gastrointestinal bleeding (p = 0.03) were significantly associated with the qualification for the antifibrotic treatment.Conclusion:This real-world prospective study showed that dose protocol and patient preference were more frequently associated with the choice of nintedanib, while the comorbidity profile and concomitant medication use were more frequently associated with the choice of pirfenidone. Age, TLCO, and history of gastrointestinal bleeding were significant factors influencing the decision to initiate antifibrotic therapy.

Barriers and facilitators to best care for idiopathic pulmonary fibrosis in Australia.

In Australia, little is known about delivery of care for people with idiopathic pulmonary fibrosis (IPF). This study examined the organization of IPF care across Australia, how it aligns with guidance for best practice, and identified barriers and facilitators to best care. Data on the organization of IPF care in Australia were collected from public hospitals using a study-specific questionnaire between February and July 2020. Semi-structured telephone interviews were conducted with respiratory physicians from around Australia between April and December 2020. Interviews were transcribed verbatim and thematic analysis was undertaken. Almost all hospitals (n= 38, 97%) held multidisciplinary meetings (MDMs) for diagnosing IPF, with 90% of multidisciplinary teams including expert respiratory physicians and radiologists; however, rheumatologists, interstitial lung disease nurses and a histopathologist were often not available. More than 90% of institutions had access to oxygen therapy, pulmonary rehabilitation and advanced care planning, but access to psychological support and clinical trials was limited (53% and 58%, respectively). Fifteen respiratory physicians (27% regional) were interviewed. Approaches to diagnosis, treatment and access to referral services were generally consistent with best practice guidance; however, regional respondents reported barriers related to inadequate staffing, lack of a nurse coordinator, inadequate access to clinical trials and funding models. Telehealth technologies were perceived as facilitators to best care. Clinical management of IPF in Australia generally aligns with best practice guidance, but there may be some inequity of access to specialist services, particularly in regional areas, that should be addressed to ensure optimal care for all.

Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be.

A significant proportion of patients with interstitial lung disease (ILD) may develop a progressive fibrosing phenotype characterized by worsening of symptoms and pulmonary function, progressive fibrosis on chest computed tomography and increased mortality. The clinical course in these patients mimics the relentless progressiveness of idiopathic pulmonary fibrosis (IPF). Common pathophysiological mechanisms such as a shared genetic susceptibility and a common downstream pathway—self-sustaining fibroproliferation—support the concept of a progressive fibrosing phenotype, which is applicable to a broad range of non-IPF ILDs. While antifibrotic drugs became the standard of care in IPF, immunosuppressive agents are still the mainstay of treatment in non-IPF fibrosing ILD (F-ILD). However, recently, randomized placebo-controlled trials have demonstrated the efficacy and safety of antifibrotic treatment in systemic sclerosis-associated F-ILD and a broad range of F-ILDs with a progressive phenotype. This review summarizes the current pharmacological management and highlights the unmet needs in patients with non-IPF ILD.

Management of idiopathic pulmonary fibrosis: A survey of Canadian respirologists

RATIONALE: International consensus guidelines provide both strong and conditional recommendations for treatment of idiopathic pulmonary fibrosis (IPF), but many recommendations are based on limited evidence. We aimed to characterize the practice patterns of IPF treatment by Canadian Respirologists and their opinions regarding the need for future clinical trials. METHODS: We conducted a cross-sectional survey of Canadian Respirologists who are members of the Canadian Thoracic Society or attended the 2018 Canadian Respiratory Conference. Survey domains were related to guideline recommendations and other challenging aspects of IPF care. We evaluated physician confidence and analyzed associations between responses and demographics. MEASUREMENTS AND MAIN RESULTS: Sixty-three Respirologists completed the survey (17% response rate). Corticosteroids were unanimously prescribed (100%) for treatment of IPF exacerbations but with substantial variability in dose and duration. Most respondents believe that placebo-controlled trials of corticosteroid treatment for IPF exacerbations are needed (92%) and ethical (83%). Pulmonary rehabilitation was always or often recommended by 67% of Respirologists; however, 25% reported poor access to exercise programs as a key barrier to participation. In patients with preserved (FVC > 90%) and severely reduced (FVC < 50%) lung function, 62% and 78% of respondents reported recommending antifibrotic therapy, respectively. For patients experiencing a decline in lung function on antifibrotics, most physicians (63%) recommend switching to the alternative antifibrotic, whereas some recommended discontinuing antifibrotic therapy (6%) or dual antifibrotics (5%). CONCLUSIONS: Despite treatment guidelines, Canadian Respirologists have diverse opinions regarding IPF treatments. This survey provides insight into the frequency of particular treatment strategies and may guide future trial design.

Impact of Idiopathic Pulmonary Fibrosis on Longitudinal Health-care Utilization in a Community-Based Cohort of Patients

Idiopathic pulmonary fibrosis (IPF) is a rare, chronic lung disease associated with substantial symptom burden, morbidity, and cost. Delivery of high-quality effective care in IPF requires understanding health-care resource utilization (HRU) patterns; however, longitudinal data from real-world populations are limited. This study aimed to define HRU attributable to IPF by evaluating a longitudinal cohort of community patients with IPF compared with matched control subjects. Incident IPF cases were identified in the Kaiser Permanente Northern California electronic health records (2000-2015) using case-validated code-based algorithms. IPF cases were compared with matched control subjects by age, sex, and length of enrollment. Annual rates of HRU measures were assessed during the 5 years pre- and postdiagnosis. Poisson generalized estimating equations were used to estimate adjusted case-control differences in HRU. IPF treatment trends were assessed before and after the availability of IPF-specific medications. A total of 691 IPF cases were identified and matched with 3,452 control subjects. Adjusted rates of all diagnostic procedures were significantly increased (P< .001) for IPF cases compared with control subjects in both the pre- and postindex periods, including chest CT scans (pre-relative risk [RR], 80.35; post-RR, 32.79), 6-min walk tests (pre-RR, 20.81; post-RR, 34.49), and pulmonary function tests (pre-RR, 9.50; post-RR, 13.24). All-cause hospitalizations (pre-RR, 1.42; post-RR, 2.33) and outpatient visits (pre-RR, 1.22; post-RR, 1.80) were significantly higher among cases compared with control subjects during both the preindex (P< .05) and postindex (P< .001) periods. We observed use of immunosuppressive and IPF-specific therapies prior to diagnosis, and high rates of corticosteroid use before and after diagnosis. This study defines a marked increase in HRU in patients with IPF compared with control subjects, with accelerated use beginning at least 1 year prediagnosis and elevated use sustained over the following 5 years. To our knowledge, this is the first study to evaluate longitudinal medication trends in IPF. Collectively, this information is foundational to advancing IPF care delivery models and supporting clinical decision-making.

A cross-sectional evaluation of the idiopathic pulmonary fibrosis patient satisfaction and quality of life with a care coordinator.

Canadian and international guidelines recommend specialized, multidisciplinary teams for the treatment of patients with idiopathic pulmonary fibrosis (IPF). The objective of this cross-sectional clinical study was to investigate the effect of a care coordinator on IPF patient satisfaction and quality of life. Forty IPF patients were enrolled from the practices of two physicians (n=20/physician), with either low (LCU) or high-coordinator use (HCU). Patient satisfaction was measured with modified FAMCARE and IPF Care UK Patient Support Program (UK-CARE) surveys. Health related quality of life (HRQoL) was assessed with the living with IPF impacts (L-IPFi) survey. An economic model assessed the impact of the coordinator; staff surveys informed patient management requirements, and costs were derived from published literature. Patient satisfaction was similar between the clinics; a trend (P=0.1) towards increased satisfaction among HCU patients was observed. Patients in the HCU clinic reported increased satisfaction (P<0.05) with their current care compared with care prior to joining the tertiary-care clinic, while LCU patients did not. IPF patient HRQoL did not differ between clinics. The coordinator was estimated to alleviate approximately 30% of a physician's IPF-related work load, and to facilitate the care of more patients per physician. Modelled estimates suggest the coordinator lead to annual cost-savings of $137,212. Reliance upon a coordinator during routine management of IPF patients may improve patient satisfaction, spare physician time and lead to annual cost-savings. Future studies should examine the impact of a coordinator on healthcare resource utilization.

Real-World Comprehensive Disease Management of Patients With Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a debilitating, progressive, and fatal fibrotic lung disease with a poor prognosis. Antifibrotic therapy slows but does not halt disease progression. Patient education and management needs change during disease progression. Management is complicated by comorbidities, adverse events associated with antifibrotic therapy, and difficulties with long-term oxygen therapy and pulmonary rehabilitation. Treating IPF requires coordination between physicians and nurses in community and interstitial lung disease center settings. This review provides guidance for the healthcare professional who manages the essential aspects of care in IPF from diagnosis, through disease progression, and to the end of life.

Understanding the patient's experience of care in idiopathic pulmonary fibrosis.

ABSTRACTBackground and objectiveNew treatments for idiopathic pulmonary fibrosis (IPF) have improved survival; however, the complexity of care may be difficult for patients. The aim of this study was to assess the patient experience of modern IPF care.MethodsA qualitative study was undertaken for 100 people with IPF with a range of disease severity (forced vital capacity range: 46–106% predicted). Participants were asked about their experience of care using semi‐structured interviews. Two investigators independently undertook analysis using the principles of grounded theory.ResultsParticipants reported dissatisfaction with information received about IPF, particularly at the time of diagnosis. Most were enthusiastic about the benefits of specialist IPF centres including regular monitoring, frequent contact with health professionals and access to clinical trials. However, the burden of travel to specialist centres and costs of treatment were significant. Many reported that side effects of anti‐fibrotics caused them to titrate or pause therapy, sometimes without consulting their treating team. Co‐morbid medical conditions had a negative impact on well‐being and highlighted poor coordination of care. Participants perceived a responsibility for self‐management that included exercise, diet, vaccination and treatment of chest infections; many felt this active role should receive more positive emphasis from health professionals.ConclusionThese data show opportunities to improve modern IPF care including accurate and timely provision of information, improving access to specialist care, more support for managing treatment side effects and better coordination of care for patients with co‐morbid conditions. Future research should explore the impact of self‐management strategies on IPF outcomes.

THE IMPACT OF ADVANCE CARE PLANNING IN INTERSTITIAL LUNG DISEASE (ILD)

SESSION TITLE: Palliative Care and End-of-Life Issues SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/10/2018 01:00 PM - 02:00 PM PURPOSE: We advocate advanced care planning (ACP) as a solution to poor acute end of life care. In 2012, we developed a multidisciplinary collaborative care model with an early integrated palliative approach to better document ACP and facilitate Idiopathic Pulmonary Fibrosis (IPF) patient wishes with respect to their care and death. We reviewed our clinic data to study the impact of ACP in IPF on the preferred location of death and on health resource utilization. METHODS: We identified 38 deceased IPF patients. Extracted data from the EMR included: demographics, duration of clinic enrollment, ACP documentation including goals of care, preferred place of care and death, dyspnea management (opiate use), actual place of death and number of hospitalizations. RESULTS: The ACP-IPF model was implemented in 100% of deceased patients, with a median follow-up period of 17.5 months. Preferred place of death was home in 74% and hospice in 13%. Actual place of death was home or hospice in 74% with a concordance rate of 85%. On average, 163 days were spent at home 6 months preceding death. 42% did not use acute care and only 16% needed 2 or more admissions. Opioids were initiated in 82% of patients, an average of 150 days preceding death. CONCLUSIONS: End of life care in IPF is marked by inadequate palliative care and increased acute care. We show that embedded within a multidisciplinary collaborative care model, ACP can be successfully implemented concurrently with routine clinical care to meet patient goals. The care design included early symptom self-management and timely coordination of needed multidisciplinary services to facilitate patient’s preferences. This is the first study to show that ACP as part of routine IPF care can improve end of life care by allowing greater adherence to patient choices, early institution of symptom treatments and decreasing acute care utilization in IPF. CLINICAL IMPLICATIONS: ACP was identified as a National Priority by the IOM 2014 report titled ‘Dying in America’, yet most IPF patients have no ACP, die in hospitals including ICUs and receive costly and inappropriate care at end of life. This the first study to our knowledge to describe the feasibility of early initiation and successful implementation of ACP in routine outpatient ILD care. It allowed mobilization of community and clinic resources and implementation of ‘dyspnea crisis plans’ within our patient centered care model, facilitating home or hospice deaths to achieve a concordance rate of 85%. Our work demonstrates improvement in end of life care by allowing greater adherence to patient choices, early institution of symptom treatments and decreasing acute care utilization in IPF. This further supports the need for wider and early ACP application in IPF and other debilitating chronic illnesses. DISCLOSURES: No relevant relationships by alia daoud, source=Web Response Speaker/Speaker's Bureau relationship with BI, ROCHE Please note: $5001 - $20000 Added 03/02/2018 by Meena Kalluri, source=Web Response, value=Consulting fee Grant relationship with Roche Please note: $5001 - $20000 Added 03/02/2018 by Meena Kalluri, source=Web Response, value=Grant/Research Consultant relationship with Boehringer Ingelheim Please note: $1001 - $5000 Added 03/03/2018 by Janice Richman-Eisenstat, source=Web Response, value=Consulting fee

Palliative and end of life care in idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis is a life-limiting interstitial lung disease. We do not know what causes it and there is no cure. People experience debilitating symptoms and delays before a diagnosis is confirmed are not uncommon. Palliative and end of life care for these patients is of variable quality. This article introduces and discusses the complexities of recognition, treatment and management of idiopathic pulmonary fibrosis, emphasising the importance of the primary care and community nurse roles. The implications for nursing practice in managing this group of people to live well and to die well will be explored.

Comprehensive and Individualized Patient Care in Idiopathic Pulmonary Fibrosis: Refining Approaches to Diagnosis, Prognosis, and Treatment

As seen in this CME online activity (available at http://courses.elseviercme.com/chest16/647e), idiopathic pulmonary fibrosis (IPF) is a specific form of chronic progressive fibrotic lung disease of unknown cause. It is the most common form of idiopathic interstitial pneumonia in adults. Its prevalence among US Medicare beneficiaries has more than doubled in the past 14 years. With the approval of two agents that reduce functional decline and disease progression, and the 2015 update of the American Thoracic Society guidelines on the treatment of IPF, the options for appropriate clinical management of the disease have become well defined. Early diagnosis and early initiation of treatment are of critical importance for long-term clinical outcomes. The diagnostic process, which may require a multidisciplinary team of experts, is centered on excluding systemic diseases and exposures and identifying a pattern of usual interstitial pneumonia on high-resolution CT or surgical lung biopsy results. Familiarity with the 2015 American Thoracic Society guidelines is critical with respect to both the treatments that the guidelines recommend and those that they advise against. Standard immunosuppressive therapy is no longer indicated, whereas pirfenidone, nintedanib, and antacid therapy are all conditionally recommended for use. Individualizing treatment is important in light of potential improved adherence to both drug therapy and health behaviors. An early referral to an interstitial lung disease center offers the advantages of comprehensive diagnostic and disease-management expertise, potential enrollment in a clinical trial, and evaluation for transplantation.

Trends in diagnosis and management of idiopathic pulmonary fibrosis in Canada

ABSTRACTRATIONALE: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a poor prognosis. Physician practice patterns on the diagnosis and management of IPF in Canada have not been described.OBJECTIVE: To describe the practice patterns of Canadian respirologists on the diagnosis and management of IPF and to determine if these patterns reflect current guidelines.METHODS: We conducted surveys of Canadian physicians and fellows-in-training in 2009 and 2013. Questions addressed the diagnosis of IPF, screening for comorbidities, treatment of IPF, and acute exacerbations of IPF.MEASUREMENTS AND MAIN RESULTS: Response rates in the 2009 and 2013 surveys were 22% and 17%, respectively. Most respondents felt that high resolution CT scan can diagnose IPF in most cases, with lung biopsy rarely performed. The majority screened for hypoxemia, pulmonary hypertension, gastroesophageal reflux, and autoimmune disease. In 2013, more respondents used no pharmacologic therapy to treat IPF (2009, 36% vs. 2013, 56%, p < 0.01) and 34% used first-line pirfenidone. Fewer respondents used prednisone (2009, 61% vs. 2013, 7%, p < 0.001) or azathioprine (2009, 38% vs. 2013, 1%, p < 0.001), while many employed first-line N-acetylcysteine (2009, 47% vs. 2013, 42%, p = 0.47). In 2013, 20% had no access to multidisciplinary diagnosis discussion, 33% accessed specialty IPF clinics, and <1% accessed palliative care. Referral for lung transplantation was higher in 2013 than 2009 (26% vs. 13%, p = 0.01). Treatment of acute exacerbations changed, with more use of corticosteroids and antibiotics, and less additional immunosuppression in 2013.CONCLUSIONS: Practice patterns for diagnosis and treatment of IPF in Canada evolved in concordance with the publication of international guidelines in 2011. Access to palliative care, lung transplantation referral, multidisciplinary diagnosis discussion, and specialty IPF clinics were identified as gaps in IPF care in Canada.

Optimizing quality of life in patients with idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a devastating, progressive and ultimately fatal lung disease. The combination of poor prognosis, uncertainty of disease course and severe symptom burden heavily impacts patients' and their families' quality of life. Though new antifibrotic drugs have been shown to decrease disease progression, the effect on health-related quality of life (HRQOL) has not been convincingly demonstrated. In a relentless disease such as IPF, striving to optimize HRQOL should complement the endeavour to prolong life. Unfortunately, there is a paucity of interventions improving symptoms and functionality for patients with IPF, and research focusing on symptom improvement, and assessing and optimizing HRQOL, is limited. This review summarizes the most recent insights into measuring and improving quality of life for patients with IPF, and discusses challenges in the management of this devastating disease. Moreover, we postulate a new model for continuous care in IPF - 'the ABCDE of IPF care': Assessing patients' needs; Backing patients by giving information and support; delivering Comfort care by focusing on treating symptoms and taking into account Comorbidities; striving to prolong life by Disease modification; helping and preparing patients and their caregivers for the eventual End-of-life events that are likely to occur.

Cardiovascular Burden and Medication Use Among Patients With Idiopathic Pulmonary Fibrosis

SESSION TITLE: Evaluation and Treatment of ILD SESSION TYPE: Original Investigation Poster PRESENTED ON: Wednesday, October 26, 2016 at 01:30 PM - 02:30 PM PURPOSE: Patients with idiopathic pulmonary fibrosis (IPF) often have a number of cardiovascular risk factors and comorbidities and take a variety of related concomitant medications. The objective of this analysis was to describe the type and prevalence of specific cardiovascular risk factors/comorbidities and related medication use in patients with IPF. METHODS: We conducted a descriptive cross-sectional analysis using a large US commercial insurance claims database to identify a cohort of patients with IPF between 2009 and 2011. We included patients with ≥ 1 inpatient claim or ≥ 2 outpatient claims with IPF (ICD-9-CM code 516.3) as one of the listed diagnoses and continuous enrollment during the identification (ID) year. Patients who had another type of interstitial lung disease after their last IPF claim in the ID year were excluded. Measures that were reported in the ID year included age, sex, health plan, usual physician specialty and cardiovascular risk factors/comorbidities and related medication use (over-the-counter medications such as aspirin were not captured). We generated descriptive results for the combined and individual ID years. RESULTS: We identified 3619 patients with IPF from 2009 to 2011: 1191 in 2009, 1292 in 2010 and 1136 in 2011. Mean (standard deviation) age was 70.4 (11.0) years, 50.5% were women, 62.6% had Medicare coverage and 49.5% received usual IPF care from a pulmonologist. Cardiovascular risk factors/comorbidities were common among patients with IPF, with ischemic heart disease being the most prevalent condition (35.7%) followed by congestive heart failure (26.8%), cerebrovascular disease (15.7%), peripheral vascular disease (11.0%), diabetes with chronic complications (9.4%), pulmonary hypertension (8.6%) and myocardial infarction (7.3%). Use of cardiovascular-related medication was evident for three-quarters of patients (74.6%): anti-hyperlipidemics (46.8%), diuretics (37.6%), renin-angiotensin inhibitors (35.5%), beta blockers (33.0%), calcium channel blockers (24.3%), anti-thrombotic agents (23.2%), anti-diabetic agents (21.6%), nitrates (9.3%), smoking cessation drugs (4.3%), anti-arrhythmic agents (3.5%) and pulmonary hypertension agents (3.0%). Results by ID year showed similar patterns. CONCLUSIONS: Cardiovascular risk factors/comorbidities and related medication use were highly prevalent among patients with IPF. These considerations should be taken into account in the overall management of patients with IPF. CLINICAL IMPLICATIONS: It is important to assess cardiovascular risk factors/comorbidities and concomitant use of related medications in the management of patients with IPF. DISCLOSURE: Karina Raimundo: Employee: Genentech, Inc. Michael Broder: Employee: Partnership for Health Analytic Research, LLC, paid by Genentech Eunice Chang: Employee: Partnership for Health Analytic Research, LLC, paid by Genentech Sheila Reddy: Employee: Partnership for Health Analytic Research, LLC, paid by Genentech Elya Papoyan: Employee: Partnership for Health Analytic Research, LLC, paid by Genentech John Stauffer: Employee: Genentech, Inc. No Product/Research Disclosure Information

Recent advances in understanding idiopathic pulmonary fibrosis.

Despite major research efforts leading to the recent approval of pirfenidone and nintedanib, the dismal prognosis of idiopathic pulmonary fibrosis (IPF) remains unchanged. The elaboration of international diagnostic criteria and disease stratification models based on clinical, physiological, radiological, and histopathological features has improved the accuracy of IPF diagnosis and prediction of mortality risk. Nevertheless, given the marked heterogeneity in clinical phenotype and the considerable overlap of IPF with other fibrotic interstitial lung diseases (ILDs), about 10% of cases of pulmonary fibrosis remain unclassifiable. Moreover, currently available tools fail to detect early IPF, predict the highly variable course of the disease, and assess response to antifibrotic drugs. Recent advances in understanding the multiple interrelated pathogenic pathways underlying IPF have identified various molecular phenotypes resulting from complex interactions among genetic, epigenetic, transcriptional, post-transcriptional, metabolic, and environmental factors. These different disease endotypes appear to confer variable susceptibility to the condition, differing risks of rapid progression, and, possibly, altered responses to therapy. The development and validation of diagnostic and prognostic biomarkers are necessary to enable a more precise and earlier diagnosis of IPF and to improve prediction of future disease behaviour. The availability of approved antifibrotic therapies together with potential new drugs currently under evaluation also highlights the need for biomarkers able to predict and assess treatment responsiveness, thereby allowing individualised treatment based on risk of progression and drug response. This approach of disease stratification and personalised medicine is already used in the routine management of many cancers and provides a potential road map for guiding clinical care in IPF.

European IPF Patient Charter: unmet needs and a call to action for healthcare policymakers.

Patient advocacy groups play an important role in supporting patients with chronic diseases and promoting better care. The aim of this patient-physician initiative was to gather perceptions from European idiopathic pulmonary fibrosis (IPF) patient advocacy groups regarding inequalities and unmet needs in IPF care, in order to develop a Patient Charter to advocate for better care.In total, 11 European patient advocacy groups were interviewed regarding the care of patients with IPF in their countries. Interview feedback was presented to a Working Group including patient advocacy group representatives and IPF specialists; key areas of agreement were developed into the European IPF Patient Charter.The interviews identified five key themes that fed into the final Charter: the need for improved diagnosis, treatment access, holistic care, disease awareness and palliative care. The final Charter was endorsed by patient advocacy groups and presented to 26 Members of the European Parliament in September 2014. It has received >8900 signatures to date.This patient-physician initiative highlights the inequalities and unmet needs in IPF care across Europe, and demonstrates how this insight can inform the development of a Patient Charter, designed as a call to action for healthcare policymakers to drive improvement in European IPF care.

IPF Care: a support program for patients with idiopathic pulmonary fibrosis treated with pirfenidone in Europe.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and irreversible fibrotic lung disease that requires long-term treatment. Given the importance of adherence to treatment and management of adverse events (AEs), patients with IPF need long-term, high-quality support in living with their condition, and adhering to therapy so they can derive maximum benefit. The IPF Care Patient Support Program (IPF Care) provides support, education, and empowerment to patients receiving pirfenidone for the treatment of IPF in Europe, through the provision of frequent, patient-managed discussions with specialist IPF nurses. In this review, we describe the structure of IPF Care in the United Kingdom (UK) and Austria, two of the longest-running IPF Care programs to date, and describe the benefits that these programs provide to patients with IPF. Analysis of results demonstrates a low rate of discontinuation from the program, and provides insight into the questions and concerns that patients express, not only with respect to pirfenidone (the only approved treatment for IPF at the time of analysis), but also in relation to other aspects of living with IPF. Pirfenidone dose modifications are common in patients in IPF Care and AEs most commonly occur early in treatment, with the majority of affected patients continuing on a stable maintenance dose. This highlights the value of the advice and support that patients receive in IPF Care regarding management of AEs and staying on treatment. Patient satisfaction was high in a survey of the UK program, with patients reporting high scores regarding ‘feeling in control of their condition’, ‘knowing what to expect from treatment’, and ‘feeling confident about how their disease is managed’. IPF Care in Europe will continue to evolve over time, striving to provide individually tailored support and patient-friendly information to improve treatment outcomes and quality of life for patients living with IPF.Electronic supplementary materialThe online version of this article (doi:10.1007/s12325-015-0183-7) contains supplementary material, which is available to authorized users.