Prevalence Of Idiopathic Pulmonary Fibrosis Research Articles

Impact of the coronavirus disease 2019 pandemic on hospital admissions for idiopathic pulmonary fibrosis: a nationwide population-based study

BackgroundPrevious studies have consistently reported a decrease in hospital admissions for respiratory diseases during the coronavirus disease 2019 (COVID-19) pandemic. However, the impact of the pandemic on idiopathic pulmonary fibrosis (IPF) admissions remains unknown.MethodsThis study used data from the Korean National Health Insurance Service database. IPF was defined based on the International Classification of Diseases 10th Revision (ICD-10) and rare intractable disease (RID) codes. The rate of IPF admissions was calculated by dividing the number of IPF admissions by the prevalence of IPF. The rate of IPF admissions during the COVID-19 pandemic (2020–2021) was compared with the mean rate of admissions during the prepandemic period (2017–2019) and presented as the rate ratio (RR). A sensitivity analysis was conducted on patients treated with systemic corticosteroids during IPF admission.ResultsIn patients with IPF defined based on the ICD-10 (analysis 1), the RRs significantly decreased from March in 2020 to December 2021, except for June and September in 2020. Similarly, in patients with IPF defined based on the ICD-10 and RID (analysis 2), the RRs significantly decreased from March 2020 to December 2021, except for June and September 2020. In the sensitivity analysis of analysis 1, the RR significantly decreased in 2020 (0.93; 95%CI: 0.88–0.99; P = 0.029), whereas the RR in 2021 was not significantly different. The RRs in the sensitivity analysis of analysis 2 significantly decreased to 0.85 (0.79–0.92; P < 0.001) in 2020 and 0.82 (0.76–0.88; P < 0.001) in 2021. In the subgroup analysis, the rates of IPF admissions significantly decreased in 2020 and 2021 across both sexes, patients aged ≥ 60 years, and all household income groups.ConclusionsThe rate of IPF admissions significantly decreased during the COVID-19 pandemic. This result indicates that preventive measures against COVID-19 may effectively mitigate IPF exacerbation. Therefore, it is assumed that there is a close relationship between respiratory viral infections and IPF exacerbations.

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

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

Incidence, prevalence and mortality of idiopathic pulmonary fibrosis in England from 2008 to 2018: a cohort study

BackgroundOwing to discrepancies in methodologies and how idiopathic pulmonary fibrosis (IPF) is diagnosed it is challenging to establish a consistent understanding of the disease burden In the UK, over 10...

A Real-World Prognosis in Idiopathic Pulmonary Fibrosis: A Special Reference to the Role of Antifibrotic Agents for the Elderly.

Idiopathic pulmonary fibrosis (IPF) is the most common and severe form of idiopathic interstitial pneumonia, and its prevalence increases with age. In the era of pre-antifibrotic agents, the median survival time of Japanese patients with IPF is 35 months, with a 5-year survival rate in western countries ranging from 20% to 40%. The prevalence of IPF is highest in elderly patients aged ≥75 years; however, the efficacy and safety of long-term use of pirfenidone and/or nintedanib are not fully understood. This study aimed to determine the efficacy and safety of the sole use of antifibrotic agents (pirfenidone or nintendanib) for IPF in the elderly. We retrospectively reviewed patients with IPF who were diagnosed and treated with either pirfenidone or nintedanib in our hospital between 2008 and 2019. We excluded patients with the subsequent use of both antifibrotic agents. We examined the survival probability and frequency of acute exacerbation, with focus on long-term use (≥1 year), elderly patients (≥75 years of age), and disease severity. We identified 91 patients with IPF (male to female ratio: 63 to 28, age 42 to 90 years). The numbers of patients with disease severity classified by JRS (I/II/III/IV) and GAP stage (I/II/III) were (38/6/17/20) and (39/36/6), respectively. The survival probabilities were comparable between the elderly (n = 46) and non-elderly groups (n = 45, p = 0.877). After the initiation of antifibrotic agents, the cumulative incidence ratio of acute exacerbation of IPF was significantly lower in the early stage (GAP stage I, n = 20) than in the progressive stage of disease (GAP stages II and III, n = 20, p = 0.028). A similar trend was noted in the JRS disease severity classification (I, II vs. III, IV) (n = 27 vs. n = 13, p = 0.072). In the long-term treatment (≥1 year) group (n = 40), the survival probabilities at 2 and 5 years after treatment initiation were 89.0% and 52.4%, respectively, which did not reach the median survival rate. Even in elderly patients (≥75 years of age), antifibrotic agents demonstrated positive effects on survival probability and the frequency of acute exacerbation. These positive effects would be improved for earlier JRS/GAP stages or long-term use.

Nationwide epidemiologic study for fibrosing interstitial lung disease (F-ILD) in South Korea: a population-based study

BackgroundFibrosing interstitial lung disease (F-ILD) is a major public health concern due to its poor prognosis. Recent clinical evidence shows that antifibrotic approaches such as pirfenidone and nintedanib provide better clinical outcome prediction in idiopathic pulmonary fibrosis (IPF) as well as selected progressive fibrosing ILD (PF-ILD) patients. Having epidemiologic insight into these diseases will be essential for the efficient utilization of these therapeutic resources. This study aimed to estimate the current prevalence, incidence, and mortality of F-ILD classified as idiopathic pulmonary fibrosis (IPF), PF-ILD other than IPF, and non-progressive F-ILD and their temporal trend in Korea.MethodsPopulation-based retrospective cohort study was conducted using the Korean Health Insurance Review and Assessment (HIRA) database (2011–2018). Patients with IPF were identified using ICD-10 code, RID code, and differential diagnosis approach. By leveraging medical records available from claim data and referencing those used in clinical trials, rigorous diagnostic criteria for PF-ILD detection were implemented.ResultsFor the past eight years, the prevalence of IPF and PF-ILD has progressively increased, while non-progressive F-ILD has remained stable. IPF, PF-ILD, and non-progressive F-ILD prevalence per 100,000 in 2018 were 16.9, 10.4, and 11.7, respectively. The incidence of IPF in 2018 was more than twice that of 2012. The incidence of PF-ILD in 2018 was 1.5 times higher than that in 2012. In 2018, the mortalites were 10.3% and 12.2% for IPF and PF-ILD, respectively. The mortality rate of PF-ILD was greater than that of IPF in all years. Unclassifiable PF-ILD and rheumatoid arthritis-PF-ILD had the highest proportion and mortality among the PF-ILD subtypes.ConclusionThe prevalence and incidence of IPF and PF-ILD have been steadily increasing in recent years. The mortality rate of PF-ILD remained consistently high and exceeded those of IPF in all years.

Pulmonary vessel volume in idiopathic pulmonary fibrosis compared with healthy controls aged > 50 years

Idiopathic pulmonary fibrosis (IPF) is characterised by progressive fibrosing interstitial pneumonia with an associated irreversible decline in lung function and quality of life. IPF prevalence increases with age, appearing most frequently in patients aged > 50 years. Pulmonary vessel-like volume (PVV) has been found to be an independent predictor of mortality in IPF and other interstitial lung diseases, however its estimation can be impacted by artefacts associated with image segmentation methods and can be confounded by adjacent fibrosis. This study compares PVV in IPF patients (N = 21) with PVV from a healthy cohort aged > 50 years (N = 59). The analysis includes a connected graph-based approach that aims to minimise artefacts contributing to calculation of PVV. We show that despite a relatively low extent of fibrosis in the IPF cohort (20% of the lung volume), PVV is 2–3 times higher than in controls. This suggests that a standardised method to calculate PVV that accounts for tree connectivity could provide a promising tool to provide early diagnostic or prognostic information in IPF patients and other interstitial lung disease.

Idiopathic pulmonary fibrosis: state of the art for 2023.

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease characterised by worsening respiratory symptoms and physiological impairment. Increasing awareness of the clinical manifestations of IPF, more widespread use of computed tomography scans and other potential factors have contributed to a rising prevalence of IPF over the last two decades, especially among people over the age of 65 years. Significant advances in the understanding of the pathobiology of IPF have emerged, and multiple genetic and nongenetic contributors have been identified. The individual patient course and the rate of disease progression in IPF are often unpredictable and heterogeneous. The rate of lung function decline is further modified by treatment with antifibrotic therapies, which have been shown to slow down disease progression. The presence of comorbid conditions may increase symptom burden and impact survival. Clinical monitoring at regular intervals to assess for disease progression by worsening symptoms, physiological parameters and/or radiological features is essential to assess the natural disease course and to guide further management, including prompt detection of complications and comorbid conditions that warrant additional treatment considerations, and timely consideration of referral to palliative care and lung transplantation for the appropriate patient. More studies are needed to determine whether early detection of IPF might improve patient outcomes. The purpose of this concise clinical review is to provide an update on IPF diagnosis, epidemiology, natural history and treatment in the context of new knowledge and latest clinical practice guidelines.

Leveraging global multi-ancestry meta-analysis in the study of idiopathic pulmonary fibrosis genetics

The research of rare and devastating orphan diseases, such as idiopathic pulmonary fibrosis (IPF) has been limited by the rarity of the disease itself. The prognosis is poor-the prevalence of IPF is only approximately four times the incidence, limiting the recruitment of patients to trials and studies of the underlying biology. Global biobanking efforts can dramatically alter the future of IPF research. We describe a large-scale meta-analysis of IPF, with 8,492 patients and 1,355,819 population controls from 13 biobanks around the globe. Finally, we combine this meta-analysis with the largest available meta-analysis of IPF, reaching 11,160 patients and 1,364,410 population controls. We identify seven novel genome-wide significant loci, only one of which would have been identified if the analysis had been limited to European ancestry individuals. We observe notable pleiotropy across IPF susceptibility and severe COVID-19 infection and note an unexplained sex-heterogeneity effect at the strongest IPF locus MUC5B.

National data on prevalence of idiopathic pulmonary fibrosis and antifibrotic drug use in Finnish specialised care

IntroductionThe previous data concerning the prevalence of idiopathic pulmonary fibrosis (IPF) and the frequency of antifibrotic drug use in Finland were based on research registries and medical records whereas nationwide...

Federal clinical guidelines on diagnosis and treatment of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic fibrotic interstitial pneumonia with a progressive course and poor prognosis. The prevalence of IPF in the Russian Federation is about 8 – 12 cases per 100,000 population. The basic treatment of IPF is antifibrotic agents.Methodology. The target audience of these clinical recommendations are therapists, general practitioners, pulmonologists, pathologists, radiologists, and medical rehabilitation doctors. Each thesis-recommendation about diagnostic and therapeutic procedures has been scored according to the scale of classes of recommendations from 1 to 5 and A, B, C scale of the levels of evidence. Clinical recommendations also contain comments and explanations to the theses, algorithms for the diagnosis and treatment of idiopathic pulmonary fibrosis, and reference materials.Conclusion. The presented clinical guidelines cover current information about the etiology and pathogenesis, clinical manifestations, diagnosis, treatment, and prevention of idiopathic pulmonary fibrosis. These guidelines were approved by the Scientific and Practical Council of the Ministry of Health of the Russian Federation in 2021.

An Overview of Herbal Medicines for Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung scarring condition with the histological characteristic of typical interstitial pneumonia. Injury to alveolar epithelial cells is a critical precursor in the pathogenesis of this disease. The prevalence of IPF is growing exponentially, with substantial morbidity and mortality rates increasing the burden on economic healthcare costs. A multidisciplinary approach for diagnosis is used to rule out the alternative causes of interstitial lung disease. Pirfenidone and nintedanib, two innovative antifibrotic medicines introduced in recent years, have provided therapeutic benefits to many IPF patients, and several IPF medications are in the early phases of clinical trials. However, available medications can cause unpleasant symptoms such as nausea and diarrhoea. More efforts have been made to uncover alternative treatments towards a more personalised patient-centred care and hence improve the outcomes in the IPF patients. Through a multi-level and multi-target treatment approach, herbal medicines, such as Traditional Chinese Medicine (TCM), have been identified as revolutionary medical treatment for IPF. Due to their natural properties, herbal medicines have shown to possess low adverse effects, stable therapeutic impact, and no obvious drug dependencies. Herbal medicines have also shown anti-inflammatory and anti-fibrotic effects, which make them a promising therapeutic target for IPF. A growing number of formulas, herbal components, and various forms of Chinese herbal medicine extracts are available for IPF patients in China. This review summarises the role of herbal medicines in the prevention and treatment of IPF.

Prevalence of idiopathic pulmonary fibrosis in Japan based on a claims database analysis

BackgroundIdiopathic pulmonary fibrosis (IPF) is a cryptogenic chronic interstitial pneumonia with progressive fibrosis and a poor prognosis. A substantial number of epidemiological studies have been conducted in Europe and the United States (US). In contrast, in Japan, only one study reported the prevalence of IPF (10.0 per 100,000 population) using clinical data (2003–2007) from one prefecture; thus, the nationwide prevalence of IPF remains unknown. This study aimed to estimate the nationwide prevalence of IPF in Japan using a nationwide claims database.MethodsWe extracted data from a Japanese claims database provided by Medical Data Vision (MDV database, April 2008–March 2019) containing data from approximately 28 million patients from 385 acute-care hospitals. Patients with IPF (those diagnosed with IPF at least once) from April 2017 to March 2018 were identified in the MDV database. The number of patients in the MDV database was extrapolated nationwide using the fourth NDB Open Data (April 2017–March 2018), and the prevalence was estimated using demographic data as denominators. The prevalence in the US, considering the same definition of IPF, was also calculated and compared with that in Japan.ResultThe number of patients with IPF in the MDV database was 4278. The estimated nationwide number of patients in Japan was estimated to be 34,040 (mean age: 73 years, percentage of men: 73%), and the prevalence was 27 per 100,000 population. In comparison with that in the US, the prevalence was similar in men and relatively lower in women until the age of 75–79 years, and it was notably lower in both sexes aged ≥ 80 years.ConclusionsWe report the nationwide IPF prevalence in Japan using data from claims databases for the first time. The prevalence estimated in this study was higher than that reported in a previous study. The difference might be due to differences in study settings and definitions of IPF. Further research should be performed to determine the prevalence more accurately and compare it with those in other countries.

Incidence, prevalence and mortality of idiopathic pulmonary fibrosis in Australia.

Idiopathic pulmonary fibrosis (IPF) is one of the most common forms of interstitial lung diseases. While studies have been conducted in other countries to determine the epidemiological burden of IPF, there is limited information in Australia. Our study aimed to address this gap and generate the first estimates for the mortality, incidence and prevalence of IPF in Australia. Estimates were generated by utilizing the novel Mortality Incidence Analysis Model (MIAMOD) method and software based on the illness-death model. Data inputs included population estimates and mortality data from the Australian Bureau of Statistics (ABS) for the period 1997-2015 and participant data from the Australian IPF Registry (AIPFR). Projections were estimated for a 10-year period up to 2025. Overall crude and age-standardized estimates for mortality were 5.9 and 6.3 per 100,000 population; incidence, 10.4 and 11.2 per 100,000 population; and prevalence, 32.6 and 35.1 per 100,000 population. Crude and age-standardized mortality, incidence and prevalence increased over the study period; however, they demonstrated a decreasing trend over the projected period. Persons older than 70 years constituted 9% of the population; however, they accounted for approximately 82%-83% of all deaths, incident and prevalent cases. All estimates were higher in males than in females. Our study provides the first estimates for incidence, prevalence and mortality of IPF in Australia. By reporting national estimates for IPF, our study addresses an information gap important for policy, planning and to help optimize the allocation of resources for the management of patients with IPF.

STUDYING THE LINK BETWEEN IDIOPATHIC PULMONARY FIBROSIS AND GI REFLUX DISEASE: A NATIONWIDE PERSPECTIVE

TOPIC: Diffuse Lung Disease TYPE: Original Investigations PURPOSE: Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial pneumonia that has been shown to have an association with gastroesophageal reflux disease (GERD) possibly due to chronic micro-aspiration leading to remodeling of the lung parenchyma. While a causal relationship between these two pathologies has not been clearly demonstrated to date, prior studies suggest they are closely related with GERD being more common in IPF than the general population. This study sought to further investigate the potential link between GERD and IPF as well as characterize sources of inpatient mortality in patients with concurrent disease. METHODS: Data from the National Inpatient Sample (NIS) database for 2015-2016 was used to assess the prevalence of IPF and the major causes of inpatient mortality in patients with both GERD and IPF. All hospitalizations with GERD (ICD-10-CM K21.0 and K21.9) were analyzed. Univariate and multivariate logistic regression analyses were performed and was adjusted for age, sex, race. SPSS was used for data analysis. RESULTS: There were 6,304,222 admissions among individuals with GERD from 2015 to 2016 with 49,870 patients also having concomitant IPF. The mean age was 72.77 years with 52.7% of patients between ages 61 to 80. Some of the comorbidities with corresponding prevalences in those with both GERD and IPF included: emphysema (2.8%), pulmonary hypertension (PAH) (17.1%), chronic obstructive pulmonary disease (COPD) (44.6%), asthma (11.3%), tobacco use (6.7%), achalasia (0.3%), and diaphragmatic hernia (5.7%). Of those with concomitant dieases, 4.2% suffered in-hospital mortality. Male sex (OR 1.190; 95% CI 1.173-1.207) was observed to have an increased risk of mortality and well as those with concomitant emphysema (OR 1.391; 95% CI 1.287-1.505), PAH (OR 1.762; 95% CI 1.709-1.817), COPD (OR 1.468; 95% CI 1.445-1.492), PE (OR 2.362; 95% CI 2.263-2.465), heart failure (OR 2.354; 95% CI 2.318-2.391) and systemic sclerosis (OR 1.737; 95% CI 1.546-1.953) but not with hypertension (OR 0.964; 95% CI 0.949-0.979), diaphragmatic hernia (OR 0.580; 95% CI 0.556-0.605), tobacco use (OR 0.441; 95% CI 0.428-0.454), and OSA (OR 0.463; 95% CI 0.448-0.479). The average length of stay was also higher in patients with GERD and coexisting IPF compared to those without IPF (4.4 days versus 5.2 days). CONCLUSIONS: Current data shows that there is an association between GERD and IPF, however, there is limited data regarding the demographics, distribution of significant comorbidities, and clinical outcomes of patients who have GERD and IPF simultaneously. Of those with both diseases, the majority of them were females above the age of 60 years and there was increased in-hospital mortality in those with concomitant emphysema, PAH, PE, heart failure and systemic sclerosis but not with hypertension, diaphragmatic hernias, diabetes or hypothyroidism. Further studies will be needed to explore how treatment of GERD in the setting of IPF may improve complication rates. CLINICAL IMPLICATIONS: Although a causal relationship between GERD and IPF has not been clearly established, our findings add to the growing knowledge that those with these simultaneous diseases are at higher risk for poor outcomes while in-hospital. DISCLOSURES: No relevant relationships by Andrew Berman, source=Web Response No relevant relationships by Konstantinos Damiris, source=Web Response No relevant relationships by Thomas Ng, source=Web Response No relevant relationships by Emily Seltzer, source=Web Response No relevant relationships by Melissa Wing, source=Web Response

Epidemiology of Idiopathic Pulmonary Fibrosis among U.S. Veterans, 2010-2019.

Rationale: The development of novel therapies for idiopathic pulmonary fibrosis (IPF) has brought increased attention to the population burden of disease. However, little is known about the epidemiology of IPF among U.S. Veterans. Objectives: This study examines temporal trends in incidence and prevalence, patient characteristics, and risk factors associated with IPF among a national cohort of U.S. Veterans. Methods: We used data from the Veterans Health Administration (VHA) electronic health record system to describe the incidence, prevalence, and geographic distribution of IPF between January 1, 2010, and December 31, 2019. We evaluated patient characteristics associated with IPF using multivariate logistic regression. Results: Among 10.7 million Veterans who received care from the VHA between 2010 and 2019, 139,116 (1.26%) were diagnosed with IPF. Using a narrow case definition of IPF, the prevalence increased from 276 cases per 100,000 in 2010 to 725 cases per 100,000 in 2019. The annual incidence increased from 73 cases per 100,000 person-years in 2010 to 210 cases per 100,000 person-years in 2019. Higher absolute incidence and prevalence rates were noted when a broader case definition of IPF was used. Risk factors associated with IPF among Veterans included older age, White race, tobacco use, and rural residence. After accounting for interactions, the average marginal difference in IPF prevalence between males and females was small. There was significant geographic heterogeneity of disease across the United States. Conclusions: This study is the first comprehensive epidemiologic analysis of IPF among the U.S. Veteran population. The incidence and prevalence of IPF among Veterans has increased over the past decade. The effect of sex on risk of IPF was attenuated once accounting for other risk factors. The geographic distribution of disease is heterogeneous across the United States with rural residence associated with higher odds of IPF. The reasons for these trends deserve further study.

Associations Between Diabetes and Idiopathic Pulmonary Fibrosis: a Study-level Pooled Analysis of 26 Million People.

Diabetes mellitus (DM) is a systemic disease characterized by chronic hyperglycemia associated with inflammation and oxidative stress, and the lung may be a target organ of diabetic microvascular damage. Several studies have indicated a positive association between idiopathic pulmonary fibrosis (IPF) and diabetes with controversial findings. Primary outcomes were to compare the prevalence of DM among individuals with IPF to non-IPF controls, and the prevalence of IPF among individuals with DM to non-DM controls. Data sources include PubMed, EMBASE, and the Cochrane Library. Studies contained sufficient data to calculate the prevalence of DM among individuals with and without IPF, or the prevalence of IPF among individuals with and without DM. Two investigators independently identified eligible studies and extracted data. Pooled odds ratio (OR) with 95% CI was the summary effect measure. Eighteen studies including 26 410 623 individuals met the eligibility criteria, of whom 16 recruited people with IPF and 2 recruited people with DM. The OR of DM in IPF patients was 1.54 (95% CI, 1.30-1.84; P < .001) compared to that in non-IPF controls. However, compared with that in non-DM patients, the risk of IPF in DM patients was not found to be significantly reduced (OR: 0.89; 95% CI, 0.64-1.25; P = .497). This meta-analysis suggests that people with IPF have 1.54 times increased odds of diabetes compared to non-IPF controls, while whether patients with DM have an increased risk of IPF is still controversial. Further large, prospective cohort studies investigating the prevalence of IPF in diabetic patients are warranted.

Global incidence and prevalence of idiopathic pulmonary fibrosis

BackgroundIdiopathic 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.MethodsA targeted literature search for population-based, observational studies reporting incidence and/or prevalence of IPF from January 2009 to April 2020 was conducted. Identified studies were aggregated by country. For countries with multiple publications, a weighted average was determined. Incidence and prevalence data were adjusted for between-study differences where possible. The final model included adjusted estimates of incidence and prevalence per 10,000 of the population with 95% confidence intervals. As prevalence estimates vary depending on the definitions used, estimates were based on a specific case definition of IPF.ResultsOverall, 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 Asia–Pacific countries, 0.09 to 0.49 in Europe, and 0.75 to 0.93 in North America. Unadjusted and adjusted incidence estimates were consistent. The adjusted prevalence estimates ranged from 0.57 to 4.51 in Asia–Pacific 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.ConclusionsDue 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 0.09–1.30 and 0.33–4.51 per 10,000 persons, respectively. According to these prevalence estimates, IPF remains a rare disease. For consistency, future epidemiologic studies of IPF should take age, sex, smoking status, and the specificity of case definitions into consideration.

Pulmonary fibrosing diseases: A short review and a therapeutic alternative

Pulmonary interstitial diseases are characterized by a wide spectrum of alterations, with idiopathic pulmonary fibrosis (IPF) being one of the most important. Recent studies have shown that COVID-19 can also progress to pulmonary fibrosis. IPF affects elderly individuals, its etiological agent is unknown and its prognosis is poor. Studies have shown an increased incidence and prevalence of IPF, especially in males. Unordered and excessive extracellular matrix deposition is the main lesion of IPF, leading to loss of normal alveolar architecture, decreased pulmonary compliance, and reduced gas exchange. Clinical trials of conventional treatments have not shown significant improvement in patients with IPF, proving the need of more effective alternatives. Studies have shown the association of angiotensin-converting enzyme (ACE)/Angiotensin (Ang)II/AT1 receptor axis with the development of pulmonary fibrosis and hypertension. On the other hand, it was observed that angiotensin 2 converting enzyme (ACE 2)/Angiotensin1-7[Ang-(1-7)]/Mas receptor axis plays an important role in the balance of the ACE/AngII/AT1 axis. In this sense, drugs that increase the activity of the ACE 2/Ang-(1-7)/Mas receptor axis could present therapeutic potential for the treatment of IPF. In addition, when the effects of ACE 2 pharmacological treatment associated with a swimming protocol were analyzed in an experimental model of bleomycin-induced lung lesions, a potent reduction of pulmonary fibrosis and an increase in endurance capacity of animals were observed. Even without fully understanding the mechanisms involved, the results of this study showed that the combination of these two treatment methods might contribute to the treatment of fibrosing interstitial lung diseases.

Efficacy of YAP1-gene Knockdown to Inhibit Alveolar-Epithelial-Cell Senescence and Alleviate Idiopathic Pulmonary Fibrosis (IPF).

The prevalence of idiopathic pulmonary fibrosis (IPF) increases with age and is associated with senescence of alveolar epithelial cells (AECs). AEC senescence in pulmonary cells mediates IPF. We herein aimed to determine if YAP1 gene knockdown, a member of the Hippo/YAP signal pathway, in the bleomycin (BLM)-induced mouse model of IPF, inhibits onset of senescence of AECs and alleviates IPF. Adeno-associated viruses (AAVs) expressing Yes-associated protein 1 (YAP1) short hairpin RNA (shRNA) were delivered into the lung of BLM-induced IPF mice via intratracheal injection, to knockdown the YAP1 gene in AECs. The mice were assigned to 4 groups: G1: control (normal mice); G2: IPF mice; G3: IPF + AAV/YAP1; G4: IPF + AAV/scramble. After 28 days, AECs were examined for senescence using H&E staining, Masson's trichrome Staining, senescence-associated ß-galactosidase (SA-β-gal) staining, western blotting and co-immunofluorescence staining, to determine the expression of YAP1, Smad-3 and p21, in order to determine the induction of senescence of ACEs. The severity of IPF determined by H&E staining, Masson's staining and immunofluorescence (IF) staining was positively correlated with the senescence of AECs. Down-regulation of YAP1 expression of the Hippo-signaling pathway, determined by western blotting in AECs, alleviated pulmonary fibrosis as determined by Masson's staining. Down regulation of YAP1 expression reduced the senescence of AECs as determined by ß-galactosidase (SA-β-gal) staining, which alleviated the clinical symptoms of IPF mice, as determined by body weight and lung index. Down-regulation of YAP1 expression in AECs inhibited AEC senescence which is thought to be the cause of IPF. Therefore, future studies can focus on inhibiting YAP1 to effectively treat IPF.

Epidemiologic Analysis of Taiwanese Patients with Idiopathic Pulmonary Fibrosis.

Several databases of epidemiologic studies in patients with idiopathic pulmonary fibrosis (IPF) have been analyzed in the Western community. However, few studies have been reported in Asia. The objective of this study was to investigate the epidemiology of IPF in Taiwan. We collected and analyzed patients with IPF from the Taiwan National Health Insurance Research Database from 2001 to 2011. We estimated the annual incidence and cumulative prevalence of IPF and mean survival time of patients and determined the causes of death. The annual incidence rates of IPF remained stable after 2005, ranging from 0.7 to 1.3 cases per 100,000 people per year, whereas the cumulative prevalence rates increased steadily from 3.1 to 6.4 cases per 100,000 people per year during 2006–2011 based on a narrow case definition. Men older than 75 years had higher incidence compared with other age groups. The mean survival after diagnosis was 6.9 years. Old age, male sex, and respiratory hospitalization were associated with shorter survival time after diagnosis. Both the incidence and prevalence rates of IPF were lower in Taiwanese patients than Western ones. Moreover, the survival time was higher in the Asian population compared with the Western population. These results may suggest the heterogeneity of the IPF definition in different study populations and geographic locations.