Materiały źródłowe

• #1 Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6313500/

  Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. Its prevalence and incidence have appeared to be increasing over the last decades. […] Determining the epidemiology of IPF is a difficult task. Overall, it makes determining and quantifying the distribution of IPF (and its determinants) in time and space a complex issue. We aim to summarize the available evidence on the incidence, mortality and burden of IPF globally and with other levels of granularity, notwithstanding the fact that the results will contain large degree of uncertainty. […] IPF accounts for 20% to half of all cases of interstitial lung disease (ILD), and represents the most frequent and severe of the idiopathic interstitial pneumonias (IIPs), a group of ILDs of unknown cause.

• #2 Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes

  https://www.mdpi.com/2076-3271/6/4/110

  Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. It is characterized by a chronic, progressive, fibrotic interstitial lung disease of unknown cause that occurs primarily in older adults. Its prevalence and incidence have appeared to be increasing over the last decades. […] Determining the epidemiology of IPF is a difficult task. Idiopathic pulmonary fibrosis shares problems and limitations with other chronic respiratory diseases, all suffering from blurred definitions, changing coding guidance and expert consensus, high under-diagnosis which often occurs very late in their natural history, as well as the status of rare disease occurrence. Overall, it makes determining and quantifying the distribution of IPF (and its determinants) in time and space a complex issue. We aim to summarize the available evidence on the incidence, mortality and burden of IPF globally and with other levels of granularity, notwithstanding the fact that the results will contain large degree of uncertainty.

• #3 SciELO Brazil – Idiopathic pulmonary fibrosis: current diagnosis and treatment Idiopathic pulmonary fibrosis: current diagnosis and treatment

  https://www.scielo.br/j/jbpneu/a/LxHMH8dXfJCpBTzC6qyH9xB/

  Epidemiological data are scarce, especially in low/medium-income countries, but its incidence and prevalence appear to be rising, reaching annual rates of more than 8 and 28 cases per 100,000 population per year, respectively. […] In Brazil, Baddini-Martinez and Pereira estimated, based on data from the USA, an annual incidence of 3.5-5.1/100,000 population and a prevalence of 7.1-9.4 per 100,000 population. […] Mortality is high, and most patients have an estimated survival of 3-5 years without treatment, which is comparable to cancers with poor prognosis.

• #4 Orphanet: Idiopathic pulmonary fibrosis

  https://www.orpha.net/en/disease/detail/2032

  Idiopathic pulmonary fibrosis (IPF) incidence appears to be increasing. Reported incidences range from 0.2 per 100.000 per year to 94 per 100.000 per year. The prevalence is estimated to be higher in men than in women. […] The median survival without treatment is 2 to 5 years from the time of diagnosis.

• #5 Epidemiology of Idiopathic Pulmonary Fibrosis | IntechOpen

  https://www.intechopen.com/chapters/77180

  Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD) classified under idiopathic fibrotic disorders of the lung. It is the most common type of ILD presenting clinically in the seventh decade of life, almost always at the later stage of illness, attributed to its earlier nonspecific presentation. […] However, accurate epidemiological data for IPF is minimal, with some being contradictory. Inconsistency in the case definition criteria and methodology has resulted in epidemiological inaccuracy when used to detect patients in the study population. […] Notable epidemiological differences are observed in the European, American, and Asian countries. Some countries have set up national registries to collect essential patient data for future studies and comparison with other countries.

• #6 Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6313500/

  Within ILD conditions, IPF accounts for 1737% of all ILD diagnoses. […] A study of USA Medicare beneficiaries aged 65 years observed IPF rates higher than previously stated (incidence of 93.7 cases per 100,000/year, with a prevalence from 202.2 cases per 100,000 in 2001 to 494.5 cases per 100,000 in 2011). […] Most concluded higher prevalence and incidence rates among men and with increasing age, especially after 75 years. […] Mortality from IPF appears to increase steadily worldwide, even considering the abovementioned overarching underestimation. […] In 2014, between 28,000 and 65,000 deaths in Europe and between 13,000 and 17,000 deaths in the United States were estimated. […] Definitively, IPF prevalence and incidence trends are on the rise. IPF incidence is universally estimated to be around 10 per 100,000 per year, and is uncommon in those younger than 50 years old and more frequent from the sixth decade on. […] More and better epidemiology might help us to better determine the burden and unmet needs of IPF, now and in the near future.

• #7 Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes

  https://www.mdpi.com/2076-3271/6/4/110

  Definitively, IPF prevalence and incidence trends are on the rise. IPF incidence is universally estimated to be around 10 per 100,000 per year, and is uncommon in those younger than 50 years old and more frequent from the sixth decade on. It is more frequent in males than females, and it can be observed in all races and ethnic origins, although in all likelihood with substantial variability.

• #8 Global incidence and prevalence of idiopathic pulmonary fibrosis | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-021-01791-z

  Idiopathic pulmonary fibrosis (IPF) is a progressive debilitating lung disease with considerable morbidity. Heterogeneity in epidemiologic studies means the full impact of the disease is unclear. A targeted literature search for population-based, observational studies reporting incidence and/or prevalence of IPF from January 2009 to April 2020 was conducted. Overall, 22 studies covering 12 countries met the inclusion criteria, with 15 reporting incidence and 18 reporting prevalence estimates. The adjusted incidence estimates (per 10,000 of the population) ranged from 0.35 to 1.30 in AsiaPacific countries, 0.09 to 0.49 in Europe, and 0.75 to 0.93 in North America. The adjusted prevalence estimates ranged from 0.57 to 4.51 in AsiaPacific countries, 0.33 to 2.51 in Europe, and 2.40 to 2.98 in North America. South Korea had the highest incidence and prevalence estimates. When prevalence estimates were compared to country-specific rare disease thresholds, IPF met the definition of a rare disease in all countries except South Korea. There were notable geographic gaps for IPF epidemiologic data. Due to differences in study methodologies, there is worldwide variability in the reported incidence and prevalence of IPF. Based on the countries included in our analysis, we estimated the adjusted incidence and prevalence of IPF to be in the range of 0.091.30 and 0.334.51 per 10,000 persons, respectively. According to these prevalence estimates, IPF remains a rare disease. Epidemiology estimates of IPF are derived using various data sources. For those using claims databases, it is important to differentiate between specific versus non-specific case definitions of IPF, as estimates can vary drastically depending on the definitions used. Single studies describing the epidemiology of IPF can also be misleading if age, sex, and other risk factors are not taken into consideration. The mean age of IPF patients is around 6570 years, with incidence increasing with age. Globally, patient numbers are rising, which may be attributed to, among other causes, an aging population, a higher degree of disease awareness and improved diagnostic tools. Overall, owing to diagnostic challenges, updated diagnostic criteria, and differences in study methodologies there is substantial heterogeneity between studies providing estimated epidemiology data in IPF, impacting the understanding of global disease burden.

• #9 Epidemiology and comorbidities in idiopathic pulmonary fibrosis: a nationwide cohort study | BMC Pulmonary Medicine | Full Text

  https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-023-02340-8

  Idiopathic pulmonary fibrosis (IPF) is frequently accompanied by comorbidities, with the management of these comorbidities crucial for clinical outcomes. This study investigated the prevalence, incidence, changes over time, and clinical impact of comorbidities in IPF patients, based on nationwide claims data in South Korea. […] The yearly prevalence rate of IPF increased from 7.50 to 23.20 per 100,000 people, and the yearly incidence rate increased from 3.56 to 7.91 per 100,000 person-years over time. […] The yearly prevalence and incidence of IPF and comorbidities in Korea increased over time. These comorbidities affected the use of pirfenidone and medical resources. […] The present utilised Korean nationwide data to evaluate the epidemiology of IPF and its associated comorbidities, and to compare the proportion of these comorbidities in groups classified by sex, age and pirfenidone use. This study found that both the prevalence and incidence of IPF in South Korea increased annually over time.

• #10 IPF: Statistics, Facts, and You

  https://www.healthline.com/health/managing-idiopathic-pulmonary-fibrosis/ipf-facts

  IPF is considered a rare, sporadic disease. According to the National Institutes of Health (NIH), about 100,000 people in the United States have IPF. Approximately 30,000 to 40,000 new cases are diagnosed each year. Worldwide, IPF affects 13 to 20 out of every 100,000 people. […] While its hard to pinpoint who exactly gets IPF, one 2016 study reported that slightly more American men are diagnosed with the disease than women. Another predictive factor is age. The NIH has reported that older age is a common diagnosis factor of IPF, with most cases diagnosed in people in their 60s or 70s. […] The most common complication that occurs in IPF is hypoxemia, or a low blood oxygen level. This often requires dependency on supplemental oxygen therapy. Exacerbations, or worsening symptoms, are another of the main complications of living with IPF. An acute exacerbation typically occurs after an infection, heart failure, or pulmonary embolism. However, an acute exacerbation may also occur without any known cause. An exacerbation can present itself as a dry cough or breathlessness. Other more serious complications can also arise, such as the development of blood clots in the lungs, high blood pressure in the heart or lungs, or even lung cancer.

• #11 Idiopathic pulmonary fibrosis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/idiopathic-pulmonary-fibrosis/

  Idiopathic pulmonary fibrosis has an estimated prevalence of 13 to 20 per 100,000 people worldwide. About 100,000 people are affected in the United States, and 30,000 to 40,000 new cases are diagnosed each year. […] Familial pulmonary fibrosis is less common than the sporadic form of the disease. Only a small percentage of cases of idiopathic pulmonary fibrosis appear to run in families.

• #12 Clinical manifestations and diagnosis of idiopathic pulmonary fibrosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-idiopathic-pulmonary-fibrosis

  Idiopathic pulmonary fibrosis (IPF) is the most common of the spontaneously occurring diffuse parenchymal lung diseases also known as idiopathic interstitial pneumonias (IIPs). […] Reported prevalence and incidence data for IPF depend on ascertainment, reporting methods, and the population evaluated. Both prevalence and incidence increase with advancing age, with presentation commonly occurring in the sixth and seventh decades; IPF is rare in patients less than 50 years of age. […] Overall, the incidence of IPF is increasing worldwide and conservative estimates range from 0.9 to 13 per 100,000 persons, and the prevalence ranges from 3.3 to 45 per 100,000 persons. […] Among a random sample of United States Medicare beneficiaries (largely ≥65 years old), the prevalence of IPF was 494 cases per 100,000, and the incidence was 94 cases per 100,000 persons per year.

• #13

  https://www.healio.com/news/pulmonology/20220228/prevalence-incidence-of-idiopathic-pulmonary-fibrosis-up-over-10-years-among-us-veterans

  In the past decade, the incidence of idiopathic pulmonary fibrosis nearly tripled and prevalence more than doubled among U.S. veterans, according to an analysis published in the Annals of the American Thoracic Society. […] This comprehensive epidemiologic analysis utilized data from 10.7 million veterans enrolled in the Veterans Health Administration electronic health record system and identified the incidence, prevalence and geographic distribution of IPF from 2010 to 2019. […] A total of 139,116 veterans had an IPF diagnosis, with IPF prevalence increasing from 276 cases per 100,000 in 2010 to 725 cases per 100,000 in 2019. In addition, the annual incidence for IPF increased from 73 cases per 100,000 person-years in 2010 to 210 cases per 100,000 person-years in 2019. […] Researchers observed higher absolute incidence and prevalence rates for IPF when using a broader case definition of the disease. […] IPF risk factors included older age, white race, tobacco use and rural residency. Researchers noted that the average marginal difference in prevalence of IPF between men and women was small, with a significantly geographic heterogeneity of disease across the U.S.

• #14 Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6313500/

  In Europe alone, approximately 40,000 new cases are diagnosed each year. […] Taking all of the above into account, IPF is also an expensive disease, with direct treatment costs of around 25,000 USD/person-year, which is a higher cost in comparison to breast cancer and many other serious conditions. […] The Global Burden of Disease (GBD) does not yet map IPF within its remit. However, IPF is included within the term interstitial lung diseases and pulmonary sarcoidosis. […] According to the GBDs tools, there is wide heterogeneity in the distribution of this category of diseases by country, with prevalence ranging 30-fold from 214.5 per 100,000 in Guam to a low of 6.8 per 100,000 in Benin; mortality ranging 150-fold from 10.5 per 100,000 in Japan to a low of 0.064 per 100,000 in Burkina Faso.

• #15 Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes

  https://www.mdpi.com/2076-3271/6/4/110

  IPF accounts for 20% to half of all cases of interstitial lung disease (ILD), and represents the most frequent and severe of the idiopathic interstitial pneumonias (IIPs), a group of ILDs of unknown cause. Idiopathic pulmonary fibrosis is considered a rare disease (occurring in less than 5 per 10,000 person-years), yet its burden is high. In Europe alone, approximately 40,000 new cases are diagnosed each year. […] Within ILD conditions, IPF accounts for 17–37% of all ILD diagnoses. However, a large variability of prevalence and incidence rates is observed in national and international studies due to a number of limitations. […] Mortality from IPF appears to increase steadily worldwide, even considering the abovementioned overarching underestimation. In 2014, between 28,000 and 65,000 deaths in Europe and between 13,000 and 17,000 deaths in the United States were estimated.

• #16 P272 Epidemiology of idiopathic pulmonary fibrosis in the uk: findings from the british lung foundation’s ‘respiratory health of the nation’ project | Thorax

  https://thorax.bmj.com/content/71/Suppl_3/A236.1

  An estimated 32,500 people in the UK live with IPF, a prevalence rate of about 50/100,000. This is more than double NICEs 2015 estimate of 1525 per 100,000. There are around 6,000 new cases diagnosed/year, greater than previous estimates of around 5,000. Overall, 5,300 people/year die from IPF, slightly more than the previous commonly accepted estimate of 5,000. There are nearly 9,000 admissions/year for IPF, accounting for around 1.3% of all admissions due to lung disease and 1.4% of all hospital bed days, despite IPF affecting less than 0.25% of people who have had a lung disease diagnosis. IPF is 50% more common in men, and killed 60% more men than women from 2008-2012. In this period 13,974 men and 8,624 women died from IPF, broadly in line with previous estimates. Incidence increases with age, around 85% of diagnoses being made in people aged over 70. Prevalence is highest in Northern Ireland, north-west England, Scotland and Wales. IPF is least common in London. Incidence is not influenced by measures of deprivation. […] Although rare, IPF is considerably more common than previously recognised and represents a small but significant burden on NHS hospital services.

• #17

  https://link.springer.com/article/10.1007/s41030-019-0087-9

  Data on the epidemiology of idiopathic pulmonary fibrosis (IPF) in Sweden are lacking. This study estimates the incidence and prevalence of IPF in Sweden, and describes the demographic and clinical characteristics and the overall survival of patients with IPF. […] The incidence of pulmonary fibrosis in C1 ranged from 10.4 to 15.4 cases per 100,000 population per year between 2001 and 2015. The prevalence increased from 15.4 to 68.0 cases per 100,000 population per year. Patients 70 years and men had a higher incidence and prevalence of pulmonary fibrosis. […] This study underscores the importance of pulmonary fibrosis as a cause of respiratory-related morbidity and mortality in Sweden. The stable incidence and increasing prevalence over time suggests longer survival. […] The incidence of pulmonary fibrosis in the Swedish population ranged from 10.4 to 15.4 cases per 100,000 population per year between 2001 and 2015; it remained stable over time. In 2015 (the last year of the study period), the incidence was 13.9 cases per 100,000 population. On the other hand, the prevalence of pulmonary fibrosis increased steadily from 15.4 cases per 100,000 population in 2001 to 68.0 cases per 100,000 population in 2015.

• #18

  https://link.springer.com/article/10.1007/s41030-019-0087-9

  The incidence of pulmonary fibrosis was higher in the older age groups and in the male population, which is generally consistent with the findings from other population-based studies in Europe and elsewhere. […] The incidence of pulmonary fibrosis remained stable over the 15 years of observation, whereas the prevalence increased steadily over time.

• #19

  https://experts.mcmaster.ca/display/publication182495

  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 the prevalence and incidence of IPF in Canada using administrative data requiring minimal extrapolation. […] For all ages, the broad prevalence of IPF was 41.8 per 100000 (14259 cases) and was higher for men. The incidence rate was 18.7 per 100000 (6390 cases) and was higher for men. The narrow prevalence was 20.0 per 100000 (6822 cases) and incidence was 9.0 per 100000 (3057 cases). The 4-year risk of death was 41.0% and the quality of life with IPF after 2years was lower than for Global Initiative for Chronic Obstructive Lung Disease stage IV chronic obstructive pulmonary disease. […] Using comprehensive national data, the prevalence of IPF in Canada was higher than other national estimates, suggesting that either IPF may be more common in Canada or that data capture may have been previously limited.

• #20 Epidemiology of Idiopathic Pulmonary Fibrosis in Northern Italy | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0147072

  Epidemiology of Idiopathic Pulmonary Fibrosis in Northern Italy […] Idiopathic pulmonary fibrosis (IPF) is the most common and severe form of idiopathic interstitial pneumonia. Despite its clinical relevance, few studies have examined the epidemiology of IPF and temporal variation in disease incidence and prevalence. Aim of the study was to investigate the prevalence, incidence and trends of IPF in Lombardy, a region with nearly 10 million inhabitants, during 2005-2010. […] The mean annual incidence rate was estimated at 2.3 and 5.3 per 100,000 person-years using NCD and GCD, respectively. IPF incidence was higher among males, and increased with age. Trend remained stable over the years. The estimated annual prevalence rate was 35.5, 22.4, and 12.6 per 100,000 person-years using GCD, BCD and NCD, respectively, and increased with age. Moreover, we observed a positive trend over the years. Using BCD and NCD, prevalence was higher among males.

• #21 Idiopathic Pulmonary Fibrosis (IPF): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/301226-overview

  Using data obtained from a large US healthcare claims database, the incidence and prevalence of idiopathic pulmonary fibrosis is higher in men aged 55 years or older, compared with women of the same age. […] Idiopathic pulmonary fibrosis mainly affects persons aged 50 years or older. Approximately two thirds of persons diagnosed with idiopathic pulmonary fibrosis are aged 60 years or older at the time of diagnosis. Using data obtained from a large US healthcare claims database, the incidence of idiopathic pulmonary fibrosis was estimated to range from 0.4-1.2 cases per 100,000 person-years for persons aged 18-34 years. However, the estimated incidence of idiopathic pulmonary fibrosis in persons aged 75 years or older was significantly higher and ranged from 27.1-76.4 cases per 100,000 person-years.

• #22 Epidemiology of Idiopathic Pulmonary Fibrosis in Northern Italy | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0147072

  The number of IPF cases identifies as prevalent in Lombardy during 2005-2010 is reported in Table 2: the results are illustrated according to the three IPF case definitions, and stratified by gender and age groups. Based on the GCD, BCD and NCD, the number of prevalent cases of IPF was 5,441 (53.8% of whom were male), 3,573 (male: 57.2%) and 2,097 (male: 56.9%), respectively. About 70% of patients were aged 65 and older, regardless of the case definition. […] Using to the GCD, the estimated average annual prevalence rate (per 100,000 person-years) was 35.51 (95% CI: 35.02-36.00), with no significant differences between genders. The prevalence of diagnosed IPF increased with increasing age, rising from 9.77 for people aged less than 55 years (95% CI: 9.46-10.08) to 143.71 for people aged 80-84 years (95% CI: 137.89-149.53). However, prevalence dropped to 99.20 (95% CI: 93.66-104.74) for people aged 85 years and older.

• #23 Idiopathic Pulmonary Fibrosis (IPF): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/301226-overview

  The age-adjusted and sex-adjusted prevalence among residents aged 50 years or older ranges from 27.9 cases per 100,000 persons (narrow-case criteria) to 63 cases per 100,000 persons (broad-case criteria). […] Whether the incidence and prevalence of idiopathic pulmonary fibrosis are influenced by geographic, ethnic, cultural, or racial factors is unclear. […] Worldwide, the incidence of idiopathic pulmonary fibrosis is estimated to be 10.7 cases per 100,000 person-years for males and 7.4 cases per 100,000 person years for females. The prevalence of idiopathic pulmonary fibrosis is estimated to be 20 cases per 100,000 persons for males and 13 cases per 100,000 persons for females. […] Epidemiologic data from large, geographically diverse populations are limited, and, therefore this data cannot be used to accurately determine the existence of a racial predilection for idiopathic pulmonary fibrosis.

• #24

  https://www.pulmonaryfibrosis.org/understanding-pff/types-of-pulmonary-fibrosis/idiopathic-pulmonary-fibrosis

  More than 250,000 Americans are living with PF and ILD. Idiopathic pulmonary fibrosis is one of the most common forms of PF. The prevalence of PF and ILD is on the rise with more than 50,000 new cases diagnosed annually. […] Although the word idiopathic means of unknown cause, we know that there are certain factors that increase the risk of receiving an IPF diagnosis. […] A family history of pulmonary fibrosis is also a risk factor, as are certain genes, such as MUC5B, TERT, TERC, DKC1, RTEL1, AKAP13, DSP, FAM13A, DPP9, and TOLLIP. […] The presence of risk factors and comorbidities provide clues that may be helpful to a physician who suspects that an ILD is present in a patient. […] Diagnosing an ILD involves many different types of information, and making an accurate diagnosis can be complicated.

• #25 Epidemiology of idiopathic pulmonary fibrosis | CLEP

  https://www.dovepress.com/epidemiology-of-idiopathic-pulmonary-fibrosis-peer-reviewed-fulltext-article-CLEP

  Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. […] Though uncommon, it is likely underappreciated both in terms of its occurrence (ie, incidence, prevalence) and public health impact (ie, health care costs and resource utilization). Incidence and mortality appear to be on the rise, and prevalence is expected to increase with the aging population. Potential risk factors include occupational and environmental exposures, tobacco smoking, gastroesophageal reflux, and genetic factors. An accurate understanding of its epidemiology is important, especially as novel therapies are emerging.

• #26

  https://www.jci.org/articles/view/60323

  Pulmonary fibrosis occurs in a variety of clinical settings, constitutes a major cause of morbidity and mortality, and represents an enormous unmet medical need. […] The annual incidence of IPF appears to be rising and is estimated at 516 per 100,000 individuals; prevalence is 1320 per 100,000. […] IPF is more common in men, and the prevalence rises dramatically with age. […] Risk factors for IPF include age, male gender, and a history of cigarette smoking. […] The importance of accurate classification of pulmonary fibrosis lies in the fact that the natural history and the potential response to treatment differ depending on the etiology. […] It is important to determine the presence or absence of an underlying connective tissue disorder because some forms of connective tissue disease-related fibrosis are reversible.

• #27 Overview of Idiopathic Pulmonary Fibrosis (IPF) and Evidence-Based Guidelines

  https://www.ajmc.com/view/overview-of-idiopathic-pulmonary-fibrosis-ipf-and-evidence-based-guidelines-article

  Conversely, an analysis of claims from US adults aged 18 to 64 years showed that the incidence of IPF decreased from 2004 to 2010, with the reduction occurring primarily in younger patients. […] Several risk factors have been linked to the development of IPF, including genetic susceptibility, environmental and occupational exposures, tobacco smoking, comorbidities (particularly gastroesophageal reflux disease [GERD]), and possible association of viral infections. […] The majority of patients with IPF have multiple comorbid conditions, which, rather than fibrotic disease itself, may contribute to the high morbidity and mortality they experience. […] Guidelines for the management of IPF address the treatment of pulmonary hypertension and GERD. […] Observational studies have demonstrated the ability of a bundled, multidisciplinary approach to improve transplant-free survival among patients with IPF. […] Lung transplantation can improve survival among patients with IPF and should be considered in patients with moderate to severe IPF.

• #28 Idiopathic Pulmonary Fibrosis Mortality by Industry and Occupation — United States, 2020–2022 | MMWR

  https://www.cdc.gov/mmwr/volumes/74/wr/mm7407a1.htm

  Idiopathic pulmonary fibrosis (IPF), a progressive lung disease characterized by scarring and worsening lung function, has a poor prognosis. An estimated 21% of IPF deaths might be attributable to occupational exposures. […] This exploratory analysis of 2020-2022 multiple cause-of-death data identified 67,843 IPF deaths among U.S. workers. By industry, IPF mortality was most significantly elevated among males employed in utilities and among females employed in public administration. By occupation, IPF mortality was most significantly elevated among male community and social services workers and among female farming, fishing, and forestry workers. […] Estimates of elevated IPF mortality among workers in some industries and occupations warrant confirmation and continued surveillance to identify occupational exposures that could be targeted to prevent or reduce IPF mortality.

• #29 Epidemiology of Idiopathic Pulmonary Fibrosis in Northern Italy | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0147072

  The results of this study, which is one of the largest population-based survey ever conducted according to strict criteria, indicated that prevalence of IPF increased across the years while incidence remained stable, thus suggesting that survival with IPF has improved. […] Epidemiology of IPF in Italy has been poorly investigated. In two studies, the prevalence of IPF has been evaluated analysing data collected in the Italian Registry of Diffuse Infiltrative Pulmonary Diseases, a multicentre prospective registry established in 1998. However, only the percentage of IPF diagnosis among patients affected by interstitial lung disease (ILD) was evaluated. Moreover, this registry was created using data obtained from those centres that voluntarily accepted to adhere to the project and, therefore, from those physicians who spontaneously provided information on number and clinical characteristics of patients with ILDs. It is likely that the use of this registry might have resulted in an underestimation of IPF cases.

• #30 Idiopathic Pulmonary Fibrosis – Epidemiology Forecast to 2029

  https://www.researchandmarkets.com/reports/5147745/idiopathic-pulmonary-fibrosis-epidemiology?srsltid=AfmBOoofVkLoLhkfSyfWYphk_b-yU1lut8Rbf9JPDK6X6n4HLlLAogHA

  Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease and is a serious chronic condition that affects the tissue surrounding the air sacs, or alveoli, in the lungs. […] The publisher epidemiologists utilized county-specific studies published in peer-reviewed journals to build the forecast. […] The report includes a 10-year epidemiological forecast for the diagnosed incident and prevalent (total and diagnosed) cases of IPF in the 7MM, segmented by age, sex, severity, and comorbidities. […] In the 7MM, the publisher’s epidemiologists forecast an increase in the diagnosed incident cases of IPF from 28,301 cases in 2019 to 32,997 cases in 2019, at an AGR of 1.66% over the forecast period. […] The diagnosed prevalent cases of IPF in the 7MM will increase from 130,822 cases in 2019 to 153,747 cases in 2029, at an AGR of 1.75% over the forecast period.

• #31 Idiopathic Pulmonary Fibrosis – Epidemiology Forecast to 2029

  https://www.researchandmarkets.com/reports/5147745/idiopathic-pulmonary-fibrosis-epidemiology?srsltid=AfmBOoofVkLoLhkfSyfWYphk_b-yU1lut8Rbf9JPDK6X6n4HLlLAogHA

  The total prevalent cases of IPF in the 7MM will increase from 201,698 cases in 2019 to 237,113 cases in 2029, at an AGR of 1.76% over the forecast period. […] IPF is more common in men, and the incidence and prevalence of IPF typically increases with advancing age. […] These trends are reflected in the publishers forecast for the diagnosed incident and prevalent (total and diagnosed) cases for the 7MM.

• #32

  https://www.sciepublish.com/article/pii/397

  IPF The annual incidence of IPF in the USA is estimated to range from 6.8 to 8.8 cases per 100,000 population. Both incidence and prevalence increase with age. IPF is more common among males and has been on the rise in recent years. The median survival for untreated patients is approximately 2 to 3 years post-diagnosis and 15 to 20% of patients experience acute exacerbations, which are typically severe and can be fatal. Additionally, IPF is more frequently reported in males, particularly those who are middle-aged, typically between 40 and 70 years old. Around two-thirds of IPF patients are over 60 years of age at the time of diagnosis. […] PPF Approximately one-third of patients with fibrosing interstitial lung disease (ILDs) other than IPF may develop PPF. The overall incidence of idiopathic non-specific interstitial pneumonia (iNSIP), regardless of the progressive-fibrosing phenotype, is lower than that of IPF. Some types of fibrosing ILDs are more likely to progress than others. For instance, PPF may be present in 53% of patients with unclassifiable interstitial lung disease (uILD), 40% of those with systemic sclerosis-associated ILD (SSc-ILD), 32% of patients with rheumatoid arthritis-associated ILD (AR-ILD), and 21% of patients with fibrotic hypersensitivity pneumonitis (HP). However, the burden of PPF and the prevalence of comorbidities are not well understood, as the diagnosis of ILDs and the recognition of PPF are often delayed, typically depending on evidence of progression obtained through regular monitoring.

• #33 Epidemiology of Idiopathic Pulmonary Fibrosis | IntechOpen

  https://www.intechopen.com/chapters/77180

  As it is a rare disease, an accurate, consistent epidemiological methodology needs to be followed for data collection to measure the incidence and prevalence of IPF. […] The consensus evidence-based guidelines in 2011 guide how to arrive at a diagnosis after ruling out other ILD causes and the need for multidisciplinary specialists input. […] The IPF incidence has increased in all studies except for two quality studies, one each from Denmark and USA. When all studies are considered, the IPF incidence ranges from 0.22 to 93.7 per 100,000 per year. […] In Europe, the higher rates were observed in the UK, while Scandinavia and Southern Europe revealed lower rates. […] Overall, increased incidence rates are observed in the UK, European, South American, and East Asian epidemiological studies.

• #34 Idiopathic Pulmonary Fibrosis | AAFP

  https://www.aafp.org/family-physician/patient-care/care-resources/respiratory-health/idiopathic-pulmonary-fibrosis.html

  While IPF is rare, a lack of large-scale studies makes it difficult to estimate the incidence of the disease. We do know that the incidence of IPF increases with age, occurring most often after 55 years, and slightly more often in men. Among all individuals 55 to 64 years old, the incidence is 19.3 cases per 100,000 person-years. […] Misdiagnosis and delays in diagnosis of IPF are common. In one study, IPF was most often misdiagnosed as asthma (13.5%), pneumonia (13.0%), or bronchitis (12.3%). Delays in diagnosis have been reported to be from one year to as long as three years, with longer delays associated with an increased risk of death. […] Diagnosing IPF is challenging, but guidelines are available to help primary care physicians identify patients with IPF and make earlier referrals. Early referral means earlier treatment, which can help improve patient outcomes.

• #35 Latin American Registry of Idiopathic Pulmonary Fibrosis (REFIPI): Clinical Characteristics, Evolution and Treatment | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-latin-american-registry-idiopathic-pulmonary-articulo-S0300289622003295

  Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and frequently fatal disease. Currently there are national and multinational registries in Europe, United States, Australia and China to better understand the magnitude of the problem and the characteristics of the IPF patients. However, there are no national or regional registries in Latin America, so the objective of this study was to carry out a Latin American registry that would allow the identification of IPF patients in our region. […] National registries, each with its own methodology, have now been created in Germany, Sweden, the United Kingdom, Greece, Spain, Australia, India and China. […] In 2014 Ryerson et al. called for the immediate establishment of a global IPF registry. […] The main objective of the REFIPI was to determine the demographic, clinical, functional and radiological characteristics of patients with IPF in Latin America at the time of diagnosis and over the course of their disease, and to record the treatments used, their efficacy and their safety in clinical practice.

• #36 Epidemiology and Natural Course of IPF

  https://www.pulmonary-fibrosis.net/index.php/european-ipf-registry-and-biobank/background-and-state-of-the-art/27-epidemiology-and-natural-course-of-ipf

  Idiopathic Pulmonary Fibrosis (IPF) is a life-threatening and devastating disease, for which no cure exists at the moment. Although epidemiological data are scarce, the prevalence of IPF has been reported to range between 20.2/100000 (men) and 13.2/100000 (women) and the incidence between 10.7/100000 (men) and 7.4/100000 (women) in a population based study in New Mexico, USA. […] Concerning Europe, IPF was found to account for approximately 20-30 % of all ILD cases and a prevalence rate of 16-18/100000 was reported. Hence, there are probably 200000 patients with IPF living in the EU. […] The average life expectancy of IPF patients upon first diagnosis still ranges only between 2-3 years, with lung transplantation being the most promising, although imperfect and inconsistently realized treatment option. Not unexpectedly, the socioeconomical burden of the disease is assumed to be high.

• #37

  https://journals.lww.com/co-pulmonarymedicine/fulltext/2022/09000/epidemiology_and_real_life_experience_in.11.aspx

  Idiopathic pulmonary fibrosis (IPF), characterized by relentless disease progression from the time of diagnosis, is part of a larger group of chronic fibrosing interstitial lung diseases (ILDs). A proportion of patients with non-IPF ILDs may develop, despite conventional treatment, a progressive pulmonary fibrosis (PPF), also referred to as ILD with a progressive fibrosing phenotype (PF-ILD). These patients experience worsening of respiratory symptoms, decline in lung function, and early mortality. The goal of this review is to describe the epidemiology and recent real-life cohorts of PF-ILD, with implications for management. […] The relatively new concept of PF-ILD has aroused active clinical research over the past years. To understand risk factors for progression and the real burden of the disease is crucial to improve management. In the last 2 years, different cohort studies have addressed these questions. They showed that almost one-third of the non-IPF fibrotic ILD patients develop PF-ILD or PPF.

• #38 Epidemiology and real-life experience in progressive pulmonary fibrosis

  https://ouci.dntb.gov.ua/en/works/73oZdX07/

  Epidemiology and real-life experience in progressive pulmonary fibrosis […] Purpose of review Idiopathic pulmonary fibrosis (IPF), characterized by relentless disease progression from the time of diagnosis, is part of a larger group of chronic fibrosing interstitial lung diseases (ILDs). A proportion of patients with non-IPF ILDs may develop, despite conventional treatment, a progressive pulmonary fibrosis (PPF), also referred to as ILD with a progressive fibrosing phenotype (PF-ILD). These patients experience worsening of respiratory symptoms, decline in lung function, and early mortality. The goal of this review is to describe the epidemiology and recent real-life cohorts of PF-ILD, with implications for management. […] Recent findings The relatively new concept of PF-ILD has aroused active clinical research over the past years. To understand risk factors for progression and the real burden of the disease is crucial to improve management. In the last 2 years, different cohort studies have addressed these questions. They showed that almost one-third of the non-IPF fibrotic ILD patients develop PF-ILD or PPF. […] Emerging data show similarities in prognosis between patients with IPF or with non-IPF PF-ILD patients. Early detection and appropriate treatment of this group of patients is a priority. Further research is needed to identify risk factors of progression, to clarify the assessment of progression in clinical practice, for a better management of patients with PF-ILD in a real-world setting.

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

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9489016/

  Data on the prevalence and incidence of ILDs other than IPF with a progressive-fibrosing phenotype are limited, in part due to the complexity and rarity of diagnosis. […] Epidemiological data relating specifically to the proportion of patients who develop progressive fibrosis within each of the differing ILDs is not very well documented; therefore, unless specified, the data considered here relate more generally to ILDs, of which a proportion of patients may present with a progressive-fibrosing phenotype. […] Despite the global distribution of ILDs with a progressive-fibrosing phenotype, their incidence and prevalence are not well defined. […] Given the negative associated prognosis, further epidemiological studies are warranted to help identify ILD patients who may develop a progressive-fibrosing phenotype and enable effective clinical management.

• #40 Epidemiology and real-life experience in progressive pulmonary fibrosis

  https://ouci.dntb.gov.ua/en/works/73oZdX07/

  A total of 215 patients were retrospectively analyzed between January 2010 and June 2023 at the Haeundae Paik Hospital in the Republic of Korea. According to the criteria proposed in 2022 by Raghu et al, PPF defined as a condition that satisfies 2 or more of the following in the past year: worsening of respiratory symptoms, physiological evidence of disease progression, and radiological evidence of disease progression. The median age of the subjects was 67 years and 63.7% were female. A total of 40% was diagnosed with PPF and connective tissue disease-associated ILD (52.3%) was the most common type, followed by nonspecific interstitial pneumonitis (NSIP) (25.6%) and cryptogenic organizing pneumonitis (16.3%). […] During the follow-up period, the mortality rate was significantly higher in the PPF group than in the non-PPF group (24.4% vs 2.3%, P < .001). In the survival analysis using the Cox proportional hazard regression model, disease progression, older age and lower forced vital capacity (FVC) were independent risk factors for mortality. Our study demonstrated that the prevalence of PPF was 40%. Concomitant therapy of steroids with an immunosuppressants and home oxygen use are risk factors for PPF. PPF itself was significantly associated with high mortality rates. Risk factors for mortality were disease progression, older age, and lower FVC.

• #41 Epidemiology and real-life experience in progressive pulmonary fibrosis

  https://ouci.dntb.gov.ua/en/works/73oZdX07/

  This real-world study expands our understanding of patients, diagnostic delays and treatment gaps experienced by patients diagnosed with PPF in Europe. There was a mean delay of 15.5 months between first ILD symptoms and ILD diagnosis. Given the progressive nature of PPF, diagnostic delay may lead to poor outcomes, including shorter survival.

• #42

  https://journals.lww.com/co-pulmonarymedicine/fulltext/2022/09000/epidemiology_and_real_life_experience_in.11.aspx

  Emerging data show similarities in prognosis between patients with IPF or with non-IPF PF-ILD patients. Early detection and appropriate treatment of this group of patients is a priority. Further research is needed to identify risk factors of progression, to clarify the assessment of progression in clinical practice, for a better management of patients with PF-ILD in a real-world setting.

• #43 Overview of Idiopathic Pulmonary Fibrosis, Evidence-Based Guidelines, and Recent Developments in the Treatment Landscape

  https://www.ajmc.com/view/overview-idiopathic-pulmonary-fibrosis-evidence-based-guidelines-recent-developments-treatment-landscape

  The approvals of pirfenidone and nintedanib substantially altered the treatment landscape of IPF; although these agents slow the decline in lung function, they do not provide a cure for IPF. […] Approximately 3 million people worldwide are living with IPF, a chronic, progressive lung disease with substantial morbidity and mortality burdens.

• #44 Epidemiology of Idiopathic Pulmonary Fibrosis | IntechOpen

  https://www.intechopen.com/chapters/77180

  The ideal sample should be large to validate the clinical diagnosis by medical records review. […] Uncertainty of diagnosis can be avoided by using internationally accepted guidelines and consolidate its use in all studies. […] National IPF registries from different countries will yield valuable data on IPF epidemiology.

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