Prevalence Of Idiopathic Pulmonary Fibrosis Research Articles

Re-evaluation of diagnostic parameters is crucial for obtaining accurate data on idiopathic pulmonary fibrosis.

BackgroundThe FinnishIPF registry is a prospective, longitudinal national registry study on the epidemiology of idiopathic pulmonary fibrosis (IPF). It was designed to describe the characteristics, management and prognosis of prevalent and incident IPF patients. The study was initiated in 2012.MethodsWe present here results limited to five university hospitals. Patients with IPF were screened from hospital registries using ICD-10 diagnosis codes J84.1 and J84.9. All patients who gave informed consent were included and evaluated using novel diagnostic criteria. Point prevalence on the 31st of December in 2012 was calculated using the reported population in each university hospital city as the denominator.ResultsPatients with ICD-10 codes J84.1 and J84.9 yielded a heterogeneous group – on the basis of patient records assessed by pulmonologists only 20–30 % of the cases were IPF. After clinical, radiological and histological re-evaluation 111 of 123 (90 %) of patients fulfilled the clinical criteria of IPF. The estimated prevalence of IPF was 8.6 cases/100 000. 60.4 % were men. Forty four percent of the patients were never-smokers. At diagnosis, the patients’ mean age was 73.5 years and mean FVC was 80.4 % and DLCO 57.3 % of predicted.ConclusionsOur results suggest that hospital registries are inaccurate for epidemiological studies unless patients are carefully re-evaluated. IPF is diagnosed in Finland at a stage when lung function is still quite well preserved. Smoking in patients with IPF was less common than in previous reports.

Health Care Utilization and Costs of Idiopathic Pulmonary Fibrosis in U.S. Medicare Beneficiaries Aged 65 Years and Older

Management of idiopathic pulmonary fibrosis (IPF) is resource-intensive. Because an increasing prevalence of IPF was found among the elderly in the United States, it is important to understand the economic burden associated with the disease in this population. To compare health care resource utilization and costs between patients with IPF and matched control subjects without IPF in Medicare, the largest U.S. payer covering the elderly. Administrative claims from a 5% random sample of Medicare beneficiaries (aged 65+) from years 2000 to 2011 were analyzed. Incident patients with IPF were identified on the basis of International Classification of Diseases, ninth revision, Clinical Modification diagnosis codes, with at least 1 year of enrollment before (preindex) and after (postindex) the first diagnosis (index date). Up to five beneficiaries without IPF were matched to each patient with IPF, based on age, sex, race, and region. Annual health care resource utilization and medical costs (excluding outpatient drug costs) during the preindex and postindex periods were compared between patients with IPF and matched control subjects. A total of 7,855 patients with IPF were matched to 38,856 control subjects. Compared with matched control subjects during the preindex period, patients with IPF had an 82% higher risk of hospitalization (28.8 vs. 15.8%), and 72% higher total medical costs ($10,124 vs. $5,888). Compared with matched control subjects during the postindex period, patients with IPF had a 134% higher risk of hospitalization (48.7 vs. 20.8%), similar increased risk of emergency room visits (39.6 vs. 17.5%), and 134% higher total medical costs ($20,887 vs. $8,932). In the U.S. Medicare population, patients with IPF incurred substantial health care resource utilization. The annual IPF-attributable medical cost to the U.S. health care system, excluding medication costs, is estimated at close to $2 billion.

Mitochondria Homeostasis Plays a Key Role in the Age‐related Susceptibility to Lung Epithelial Injury and Fibrosis

RationaleAging is associated with increased susceptibility to lung pathologies. The prevalence of idiopathic pulmonary fibrosis (IPF) has been found to increase with age. While familial disease has been related to disordered telomerase activity and mutations of SPC, the effects of advancing age and lung epithelium dysfunction and fibrosis remains unclear.MethodsLung fibrosis and mitochondria function, morphology and dynamics were analyzed in alveolar epithelial cell type II (AECII) of patients with IPF, aging mice and mice deficient in PINK1 (PTEN‐induced putative kinase 1).ResultsWe found that AECII in IPF lung have marked accumulation of dysmorphic and dysfunctional mitochondria. These mitochondrial abnormalities were recapitulated in normal mice with advancing age and ER stress stimulation. We found that impaired mitochondria in IPF and aging lungs were associated with low expression of the PINK1. Knockdown of PINK1 expression in lung epithelial cells resulted in mitochondria depolarization, and expression of pro‐fibrotic factors. Moreover, young mice deficient in PINK1 develop similar dysmorphic dysfunctional mitochondria in the AECII and are highly vulnerable to apoptosis and development of lung fibrosis.ConclusionOur data indicates that PINK1 deficiency and the subsequent swollen mitochondria dysfunction in the presence of ER stress causes defective mitophagy and promotion of fibrosis in the aging lung.

Does hepatitis C virus enhance prevalence of idiopathic pulmonary fibrosis and affect its severity? An Egyptian study

Abstract Background Idiopathic pulmonary fibrosis (IPF) is the most common and severe form of pulmonary fibrosis characterized by gradual loss of pulmonary function. Despite rigorous research, the etiology of IPF has remained unknown. Viruses, for example, hepatitis C virus (HCV), had been implicated in IPF etiology; however, data on the prevalence of HCV infection in IPF patients were limited. Aim of the study Our aims were to assess the prevalence of HCV antibodies in IPF patients and to assess the relationship between severity of pulmonary and hepatic dysfunction. Materials and methods IPF patients were prospectively enrolled from Chest Department, Assiut University Hospital. HCV antibodies were detected using the third-generation enzyme-linked immunosorbent assay. Patients’ pulmonary and hepatic functions were evaluated. Results HCV antibodies were significantly higher in IPF patients than in controls (29.4 vs. 14%, P = 0.04). Patients with HCV had significantly more severe hypoxemia and lower diffusing capacity for carbon monoxide than those without HCV (47.7 ± 11.3 vs. 54 ± 18.7, P = 0.03 and 52.7 ± 8.4 vs. 67.3 ± 9.5, P = 0.01, respectively). There was no significant difference between HCV-positive IPF patients and HCV-negative IPF patients regarding spirometric parameters and liver function parameters. Conclusion This higher prevalence of HCV and its effect on pulmonary functions in IPF patients may contribute in IPF pathogenesis, which hopefully will allow currently available antiviral drugs or novel therapeutic approaches to treat or modify the course of this devastating disease.

P246 Lung Transplantation For Patients With Idiopathic Pulmonary Fibrosis And Asymptomatic Coronary Artery Disease

Introduction and objectives Lung transplantation significantly improves the survival of patients with advanced idiopathic pulmonary fibrosis (IPF). Concurrent coronary artery disease (CAD) is a relative contraindication to transplantation and can limit access to this therapy. This is particularly relevant as a high prevalence of CAD has been reported in patients with IPF.1 We sought to determine whether the presence of asymptomatic CAD impacted upon post-transplant survival. Methods This retrospective study reviewed all patients who had undergone single lung transplantation for IPF at our centre, between May 2005 to April 2014. We compared post-surgical outcomes for patients with IPF who had an abnormal coronary angiogram (at the time of transplant listing), to those with normal angiography. Kaplan-Meier curves were created to study survival and univarate analysis performed using the Log-Rank score. Results In this timeframe, 39 patients underwent lung transplantation for IPF, of which 22 patients (56.4%) had abnormal coronary angiography. Eight of these patients had minor disease, 5 had 10–30% stenosis and 5 had 30–50% stenosis. One patient required coronary artery stenting prior to transplantation, but 3 patients with ≥70% stenosis had no inducible ischaemia on dobutamine stress testing and were managed conservatively. All patients had normal pre-operative left ventricular function. The post-transplant survival of patients with IPF and CAD was 95.7% at 1 year and 70.1% at 5 years. There was no significant difference in survival (p = 0.52) between the cohort with CAD and those with normal pre-operative coronary angiograms. Of note, 1 patient required coronary artery stenting 18 months after transplantation (this patient had 30% LAD stenosis at time of listing). No patients developed chronic arrhythmias, and no patients died as a result of cardiovascular disease. Conclusion Although CAD remains a relative contraindication to transplantation, the effects of previous PCI or minor CAD are unknown and may be overstated. This finding is particularly relevant for patients with IPF, who may have an increased prevalence of CAD. Reference Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006;174(7):810-6

Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001–11

Published data for the epidemiology of idiopathic pulmonary fibrosis in the USA are scarce. We sought to estimate the incidence, prevalence, and mortality risk of idiopathic pulmonary fibrosis among US Medicare beneficiaries. We used administrative claims from a 5% random sample of Medicare beneficiaries (aged 65 years and older) from the years 2000-11 as a data source. Idiopathic pulmonary fibrosis was defined by International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes. We estimated annual incidence and cumulative prevalence of idiopathic pulmonary fibrosis, median survival time of patients, and potential risk factors for diagnosis of idiopathic pulmonary fibrosis and death between 2001 and 2011. We also estimated incidence and prevalence using more restrictive algorithms for diagnosis. The annual incidence of idiopathic pulmonary fibrosis in the Medicare population remained stable between 2001 and 2011, with an overall estimate of 93.7 cases per 100000 person-years (95% CI 91.9-95.4) across the study period. The annual cumulative prevalence increased steadily from 202.2 cases per 100000 people in 2001 to 494.5 cases per 100000 people in 2011. Among newly diagnosed patients with Medicare (mean age 79.4 years [SD 7.2], 54% female, 91% white), the median survival time was 3.8 years (95% CI 3.5-3.8). Older age and male sex were associated with a higher incidence of disease and shorter survival time after diagnosis. Mortality risk was lower in patients diagnosed in more recent years (median survival time 3.3 years [95% CI 3.0-3.8] in 2001 vs 4.0 years [3.8-4.5] in 2007). The incidence and prevalence of idiopathic pulmonary fibrosis in people aged 65 years and older in the USA are substantially higher than previously reported, and prevalence is increasing annually, even in the subgroups based on more restrictive algorithms for diagnosis. Patients with idiopathic pulmonary fibrosis aged 65 years and older were living longer in 2011 than they were 10 years before, which could partly account for the steady increase in prevalence. Biogen Idec.

Adding Insult to Injury: Complications of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a distinctive form of chronic interstitial lung disease occurring primarily in older adults, more often male [Raghu G et al. Am J Respir Crit Care Med 2011]. The estimated prevalence of IPF is 14 to 43 per 100,000 and an estimated incidence is 7 to 16 per 100,000, depending on how it is defined [Raghu G et al. Am J Respir Crit Care Med 2006]. Prognosis can be predicted by the radiographic extent of the disease, amount of fibrosis, presence of pulmonary hypertension, and comorbidities (emphysema, lung cancer, and coronary artery disease).

Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial pneumonia of unknown aetiology. It is a rare disease, and its incidence and prevalence are not clear. Therefore, we sought to review the published evidence on the global epidemiology of IPF. A comprehensive review of English language literature was performed by searching Medline and EMBASE for studies on IPF epidemiology published between January 1990 and August 2011. Studies providing quantitative data on IPF incidence and/or prevalence were identified and key data collected. 15 studies reporting on the incidence and/or prevalence of IPF were identified and summarised. IPF prevalence estimates in the USA varied between 14 and 27.9 cases per 100,000 population using narrow case definitions, and 42.7 and 63 per 100,000 population using broad case definitions. In Europe, IPF prevalence ranged from 1.25 to 23.4 cases per 100,000 population. The annual incidence of IPF in the USA was estimated at 6.8-8.8 per 100,000 population using narrow case definitions and 16.3-17.4 per 100,000 population using broad case definitions. In Europe, the annual incidence ranged between 0.22 and 7.4 per 100,000 population. IPF prevalence and incidence increase with age, are higher among males and appear to be on the increase in recent years. IPF is an orphan disease that affects a potentially increasing number of people in Europe and the USA. The observed variability in IPF incidence and prevalence may be explained by the differences in diagnostic criteria used, case definition, study population and study design.

Silent gastro-oesophageal reflux and microaspiration in IPF: mounting evidence for anti-reflux therapy?

Idiopathic pulmonary fibrosis (IPF) is a distinct clinical entity that generally affects people who are over 60 yrs of age, and median survival ranges from 3 to 5 yrs after the diagnosis is ascertained. The natural course of IPF is well known: the majority of patients with IPF display steady decline in lung function, some patients remain stable for prolonged periods of time, a subgroup of patients rapidly decline, and a subset of patients manifest acute exacerbations of IPF preceding death [1]. While several key cellular and molecular events that are thought to follow “injury” have been identified in the pathogenesis of IPF [2], the ultimate cause of IPF and the triggering factor(s) that injure the lung remain elusive, and none of the currently available pharmacological agents have demonstrated improved outcomes and survival in patients with IPF. The incidence and prevalence of IPF is highly linked to advanced age with an estimated incidence and prevalence of 71 and 271 per 100,000 per year for males and 67 and 266 per 100,000 per year for females aged 75 yrs or greater versus an overall incidence and prevalence of 16.3 and 42.7 per 100,000 per year using broad diagnostic criteria [3]. Interestingly, many observations suggest that the aged lung is more susceptible to injury and fibrosis induced by a variety of stimuli, and this susceptibility may be linked to age-associated changes in gene expression or genetic polymorphisms such as age-associated telomerase dysfunction [4–7]. Advanced age is also accompanied by a decline in oesophageal and gastric motility, diminished upper oesophageal sphincter pressure, and an increase in oesophageal acid exposure [8]. Hiatal hernias appear with advancing age and have been detected in up to 60% of individuals older than 60 yrs [ …

Living with idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is the most common of the seven listed interstitial pneumonias in the American Thoracic Society/European Respiratory Society (ATS/ERS) consensus statement, and there is growing evidence that the incidence is increasing. Defined as a progressive fibrosing interstitial pneumonia of unknown cause, IPF is defined as being limited to the lungs and associated with histopathological and/or radiographical patterns consistent with usual interstitial pneumonia (UIP). A recent article by Navaratnam and colleagues documents the prevalence in UK to be around 15,000, with >5000 new cases per annum and unfortunately around 5000 deaths. With a median survival of 3–5 years after diagnosis, this is a devastating disease although the natural course of the disease varies, with some patients experiencing a steady decline over a few years, some experiencing a rapid decline, and others experiencing what appear to be similar to acute exacerbations in between relative periods of stability. Unlike the more common respiratory disorders such as chronic obstructive pulmonary disease (COPD) where patients are likely to die of other causes than the underlying COPD, those diagnosed with IPF will most often die from their disease process, estimated at around 60%, rather than with it and often during acute exacerbations. Mortality in patients with IPF increases with advancing age. Death rates are consistently higher in men than women, and there is seasonal variation, with the highest death rates occurring in the winter, even with the exclusion of infectious causes. If the cause of death is not attributable to an acute exacerbation of IPF, there are increased cardiovascular risks and an increased risk of venous thromboembolic disease which contribute to the cause of death. Other common causes of death in patients with IPF therefore include acute coronary syndromes, congestive heart failure, lung cancer, and infectious causes. Regrettably, late presentation often shortens the survival time of patients with IPF and there is often a delay of 1–2 years before presentation with symptoms and additionally there may be a problem with clinicians missing early clinical clues, although this will not alter the natural course of the disease process. The epidemiological origins and subsequent pathological developments of the process of IPF are still not completely understood, although the condition is believed to result from an inflammatory response to an unknown substance resulting in pathological scarring, and there may also be epidemiological and genetic factors. Whether the incidence and prevalence of IPF are influenced by geographic, ethnic, cultural, or racial factors is not yet clear. The ‘idiopathic’ nature of the disease meaning no cause can be found, can be particularly difficult to both understand and accept for those diagnosed. Although the disease occurs most often in people between 50 and 70 years of age, it can affect people of a younger age, and although the preponderance is in males, it is no respecter of gender. Unfortunately for people diagnosed with the condition, IPF is often a poorly understood condition with little knowledge and unfortunately no definitive treatment leaving people diagnosed with the disorder. Available information, for example, on the Internet can be distressing and documents lack of treatment and poor prognosis. Studies to date have yet to find robust evidence for any effective pharmacological treatment of IPF. Corticosteroids and other immunosuppressants are commonly tried treatments in IPF, although unfortunately ‘tried’ is the most important word here as there is little evidence that any of these are truly effective and the side effects can outweigh any potential benefit. Any decision to attempt to treat IPF needs an open and honest discussion with patients and their views taken

Idiopathic Pulmonary Fibrosis: Update on Genetic Discoveries

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic disease of the lungs that increases in prevalence with advanced age. Recent evidence indicates that mutations in genes of two different biologic pathways lead to the common phenotype of familial pulmonary fibrosis (FPF) and sporadic IPF. Mutations in the genes encoding the lung surfactant proteins C and A2 (SFTPC and SFTPA2, respectively) cause increased endoplasmic reticulum stress in type II alveolar epithelial cells. Mutations in the genes encoding telomerase (TERT and TERC) cause IPF through shortening of telomere lengths and probable exhaustion of lung stem cells. All of the mutations are individually rare, but, collectively, TERT mutations are the most common genetic defect found in FPF. The overall penetrance of pulmonary fibrosis in TERT mutation carriers is 40% in subjects with a mean age of 51 years. Penetrance increases with advanced age, is greater in males than in females, and is positively associated with fibrogenic environmental exposures. Short telomere lengths are found in patients with FPF and sporadic IPF without mutations in telomerase, suggesting that the biologic pathway of telomerase dysfunction provides a biologic explanation for the age-related prevalence of IPF. The molecular data of two seemingly unrelated biologic pathways-alveolar epithelial endoplasmic reticulum stress and telomerase dysfunction-are beginning to elucidate the pathogenesis of IPF. These results have potentially predictive and therapeutic value.

Cecil Hougie

Idiopathic pulmonary fibrosis (IPF) is a rare disease, with estimates of prevalence varying considerably across countries due to paucity in data collection. The aim of this study was to investigate...

Incidence and Prevalence of Idiopathic Pulmonary Fibrosis

Incidence and Prevalence of Idiopathic Pulmonary Fibrosis

Incidence and Prevalence of Idiopathic Pulmonary Fibrosis

Incidence and Prevalence of Idiopathic Pulmonary Fibrosis

Incidence and Prevalence of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis is a chronic interstitial lung disease of unknown etiology; its epidemiology in the United States has not been well characterized. To estimate the annual incidence and prevalence of idiopathic pulmonary fibrosis in the United States. Retrospective cohort design utilizing a large health care claims database spanning the period January 1996 through December 2000. Persons with idiopathic pulmonary fibrosis were identified based on diagnosis and procedure codes. Using broad case-finding criteria, prevalence was estimated to range from 4.0 per 100,000 persons aged 18 to 34 yr to 227.2 per 100,000 among those 75 yr or older; annual incidence was estimated to range from 1.2 to 76.4 per 100,000. Using narrow case-finding criteria, prevalence ranged from 0.8 to 64.7 per 100,000 persons; comparable figures for incidence were 0.4 to 27.1 per 100,000 persons. Extrapolating these rates to the overall United States' population, prevalence was estimated to be 42.7 per 100,000 (incidence, 16.3 per 100,000) using broad criteria; with narrow criteria, prevalence was estimated to be 14.0 per 100,000 (incidence, 6.8 per 100,000). Our results suggest that idiopathic pulmonary fibrosis is probably more common in the United States than previously reported.

The polymorphism C5507G of complement receptor 1 does not explain idiopathic pulmonary fibrosis among the Finns

Idiopathic pulmonary fibrosis is the most common of the idiopathic interstitial lung diseases referring to the histo-pathological entity of usual interstitial pneumonia. It has been hypothesized that inflammation may trigger the multiformic fibrotic lesions found in the affected lung, and defects in the innate immune defense, including the complement, can predispose to pulmonary fibrosis. The polymorphism C5507G in the Complement Receptor 1 gene has been recently associated with idiopathic pulmonary fibrosis. C5507G causes an amino acid change from proline to arginine, and opens a potential cleavage site for trypsin-like enzymes and, therefore, a potential mechanism for increased shedding of the molecule from the cell surface. We studied the polymorphism in 96 Finnish patients with idiopathic pulmonary fibrosis and 164 population based controls. All the patients and controls were C5507 homozygous suggesting that either the Finns do not carry the G5507 polymorphism or it is extremely rare. We conclude that G5507 is not a susceptibility allele for idiopathic pulmonary fibrosis among Finnish patients.

A clinical study of idiopathic pulmonary fibrosis based on autopsy studies in elderly patients.

The prevalence of idiopathic pulmonary fibrosis (IPF) tends to be greater in the elderly although the precise outcome is not known. The present study investigated the outcome of IPF in elderly patients whose pathological diagnosis corresponded to usual interstitial pneumonia (UIP) on autopsy findings. In a consecutive autopsy series, (n = 4,554 cases), complete information was obtained in 86 cases in which the clinical and pathological diagnosis was IPF and UIP, respectively. Mean subject age was 80.5 years and there were 52 males and 34 females. Median survival time (MST) was 3.5 years overall. Dyspnea ranking by the MRC dyspnea scale (DR) on admission was closely related to the outcome. When MST was compared between DR0 and DR1, or DR2 and DR3, the former patient group survived significantly longer than the latter (p < 0.05). Twenty percent of patients died of severe respiratory failure. Other causes of death were disease progression of IPF (10.5%), bacterial pneumonia (23.3%) and lung cancer (17.4%). The patients in the young-elderly group (age < 75) survived for a significantly longer period than those in the old-elderly group (age > or = 75) (MST; 5.5 vs 2.0 years; p < 0.001). Both lung cancer and pneumonia were significantly more prevalent among IPF patients than among non-IPF patients; these IPF with lung cancer profoundly reflected the smoking history of IPF patients. The poor outcome in IPF in elderly patients was characterized by more severe dyspnea, advanced age and co-morbidity.

Nationwide prevalence of sporadic and familial idiopathic pulmonary fibrosis: evidence of founder effect among multiplex families in Finland

Background: The prevalence of sporadic and familial idiopathic pulmonary fibrosis (IPF) cases in Finland was evaluated according to the revised recommendations of the American Thoracic Society. Methods: All Finnish pulmonary...

Transverse Arrest of Head

<b>Background:</b> There is limited epidemiological data for idiopathic pulmonary fibrosis(IPF) in Australia. <b>Aim:</b> Our study aimed to estimate the mortality, incidence, and prevalence rates of IPF in Australia for the period 1997-2015 and projections for 2016-2025. <b>Methods:</b> Our estimates were generated&nbsp;utilising the&nbsp;Mortality Incidence Analysis Model (MIAMOD) method and software. This method is useful in situations where incidence and prevalence data are limited but mortality and registry data are available. Data from the Australian IPF Registry and population and death index data from the Australia Bureau of Statistics were used&nbsp;to generate&nbsp;estimates. Age standardised rates were based on the standard Australian population in 2011. <b>Results:</b> There was an increasing trend in crude and age standardised mortality, incidence, and prevalence over the study period (1997-2015), however age standardised mortality, incidence and prevalence showed a decreasing trend over the projected period from 2017-2025. Highest crude and age standardised estimates were in persons 70 years and above and all estimates were higher in males than in females. Overall crude and age standardised estimates were mortality, 5.9 and 6.2 per 100,000&nbsp;persons respectively; incidence, 10.4 and 11.0 per 100,000 person years, respectively; and prevalence, 32.6 and 34.3 per 100,000&nbsp;persons, respectively. <b>Conclusion:</b> Our study has revealed an increasing trend in mortality, incidence, and prevalence of IPF in Australia. This adds to the current evidence base for policy and planning decisions particularly related to resources for the management of patients with IPF. Moreover, this study serves as a benchmark for future research which will improve the knowledge base on IPF in Australia.