scholarly journals All-cause mortality of patients with idiopathic pulmonary fibrosis: a nationwide population-based cohort study in Korea

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Sung Ko ◽  
Sun Mi Choi ◽  
Kyung-Do Han ◽  
Chang-Hoon Lee ◽  
Jinwoo Lee

AbstractMost epidemiologic studies of patients with idiopathic pulmonary fibrosis (IPF) have been conducted in North America and Europe. Moreover, there are limited data concerning the cause of death and cause-specific mortality rate of IPF patients in population-based studies. We analyzed information from the Korean National Health Insurance Service database from 2006 to 2016. Patients with a diagnosis code of IPF were extracted from the database and we included those who satisfied the narrow definition of IPF diagnosis. Age- and sex-matched controls were randomly selected at a case-to-control rate of 1:3. We included 42,777 patients newly diagnosed with IPF during the study period. Their mean age was 64.6 years, and 65.4% were male. The age-standardized mortality rates were 85.66 (95% confidence interval [CI] 84.45–86.89) per 1000 person-years. The survival rates of IPF patients 1, 2, 3, 5, and 10 years after IPF diagnosis were 84.5%, 77.4%, 71.9%, 62.9%, and 48.4%, respectively. The standardized mortality ratio of IPF patients compared to that of the normal population was 4.66. The leading cause of death in IPF patients was respiratory causes, followed by cancer. Patients with IPF in Korea showed significantly higher mortality compared to the general population.

Author(s):  
Alessandro Marcon ◽  
Elena Schievano ◽  
Ugo Fedeli

Mortality from idiopathic pulmonary fibrosis (IPF) is increasing in most European countries, but there are no data for Italy. We analysed the registry data from a region in northeastern Italy to assess the trends in IPF-related mortality during 2008–2019, to compare results of underlying vs. multiple cause of death analyses, and to describe the impact of the COVID-19 epidemic in 2020. We identified IPF (ICD-10 code J84.1) among the causes of death registered in 557,932 certificates in the Veneto region. We assessed time trends in annual age-standardized mortality rates by gender and age (40–74, 75–84, and ≥85 years). IPF was the underlying cause of 1310 deaths in the 2251 certificates mentioning IPF. For all age groups combined, the age-standardized mortality rate from IPF identified as the underlying cause of death was close to the European median (males and females: 3.1 and 1.3 per 100,000/year, respectively). During 2008–2019, mortality rates increased in men aged ≥85 years (annual percent change of 6.5%, 95% CI: 2.0, 11.2%), but not among women or for the younger age groups. A 72% excess of IPF-related deaths was registered in March–April 2020 (mortality ratio 1.72, 95% CI: 1.29, 2.24). IPF mortality was increasing among older men in northeastern Italy. The burden of IPF was heavier than assessed by routine statistics, since less than two out of three IPF-related deaths were directly attributed to this condition. COVID-19 was accompanied by a remarkable increase in IPF-related mortality.


2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Toby M. Maher ◽  
Elisabeth Bendstrup ◽  
Louis Dron ◽  
Jonathan Langley ◽  
Gerald Smith ◽  
...  

Abstract Background 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. Methods A 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. Results 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 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. Conclusions 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 0.09–1.30 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.


Thorax ◽  
2016 ◽  
Vol 71 (Suppl 3) ◽  
pp. A13.1-A13
Author(s):  
V Navaratnam ◽  
AW Fogarty ◽  
T McKeever ◽  
N Thompson ◽  
G Jenkins ◽  
...  

2021 ◽  
Author(s):  
Nicholas T Vozoris ◽  
Andrew S Wilton ◽  
Peter C Austin ◽  
Tetyana Kendzerska ◽  
Clodagh M Ryan ◽  
...  

Abstract Background: It is not well-known if diagnosing and treating sleep breathing disorders among individuals with idiopathic pulmonary fibrosis (IPF) improves health outcomes. We evaluated the association between receipt of laboratory-based polysomnography (which is the first step in the diagnosis and treatment of sleep breathing disorders in Ontario, Canada) and respiratory-related hospitalization and all-cause mortality among individuals with IPF.Methods: We used a retrospective, population-based, cohort study design, analyzing health administrative data from Ontario, Canada, from 2007-2019. Individuals with IPF were identified using an algorithm based on health administrative codes previously developed by IPF experts. Propensity score matching was used to account for potential differences in 41 relevant covariates between individuals that underwent polysomnography (exposed) and individuals that did not undergo polysomnography (controls), in order minimize potential confounding. Respiratory-related hospitalization and all-cause mortality were evaluated up to 12 months after the index date. Results: Out of 5044 individuals with IPF identified, 201 (4.0%) received polysomnography, and 189 (94.0%) were matched to an equal number of controls. Compared to controls, exposed individuals had significantly reduced rates of respiratory-related hospitalization (hazard ratio [HR] 0.43, 95% confidence interval [CI] 0.24-0.75), p=0.003) and all-cause mortality (HR 0.49, 95% CI 0.30-0.80), p=0.004). Significantly reduced rate of respiratory-related hospitalization (but not all-cause mortality) was also observed among those with >=1 respiratory-related hospitalization (HR 0.38, 95% CI 0.15-0.99) and systemic corticosteroid receipt (HR 0.37, 95% CI 0.19-0.94) in the year prior to the index date, which reflect sicker subgroups of persons. Conclusions: Undergoing polysomnography was associated with significantly improved clinically-important health outcomes among individuals with IPF, highlighting the potential importance of incorporating this testing in IPF disease management.


Medicina ◽  
2012 ◽  
Vol 48 (3) ◽  
pp. 20 ◽  
Author(s):  
Jaunius Kurtinaitis ◽  
Jolanta Dadonienė ◽  
Giedrius Kvederas ◽  
Narūnas Porvaneckas ◽  
Tomas Butėnas

Background and Objective. To identify the survival and standardized mortality ratio with respect to gender, age, and treatment method of patients treated for femoral neck fractures. Material and Methods. A retrospective review of medical records of 736 patients treated for femoral neck fractures at Vilnius University Emergency Hospital during 2004–2006 was carried out. Results. The overall 1- and 2-year survival rates were 77.4% and 67.1%, respectively. Lower survival rates were observed in the internal fixation group than in the primary and secondary total hip arthroplasty groups (63.2% vs. 72.0% and 75.1%). Cox proportional hazards model analysis showed patient age to be a significant risk factor for survival (hazard ratio, 1.05; 95% CI, 1.04– 1.07; P<0.001). The overall standardized mortality ratio was 2.50. The standardized mortality ratios for men and women were 3.07 and 2.27, respectively, but the difference between these groups was not significant. Conclusions. Standardized mortality and survival rates decreased with increasing patients’ age. Significantly lower survival rates were documented in the internal fixation group as compared with primary and secondary total hip arthroplasty groups. There was a trend toward a higher standardized mortality ratio in men than women, but the difference was not significant.


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 6619-6619
Author(s):  
G. F. Beadle ◽  
N. J. McCarthy ◽  
P. D. Baade

6619 Background: Survivorship research after a breast cancer (BC) diagnosis has identified psychosocial, functional and comorbidity outcomes that adversely influence daily life. Little is known, however, about non-breast cancer (NBC) mortality after a BC diagnosis. Methods: This retrospective cohort study included Australian women aged 30–79 years diagnosed with BC between January 1982 and December 2004, with mortality examined from January 1993 to December 2005. The age-standardized mortality ratio (SMR) was used to compare NBC causes of death in the BC cohort and the age-matched Australian female population between January 1993 and December 2005. Results: The median follow-up time for the cohort of 159,550 Australian women diagnosed with BC was 7.04 years (range 0–24.7 years). During the follow-up period, 30,819 (19.3%) died of BC, 5,403 (3.4%) died of other cancers (OC), 18,349 (11.5%) died of non-cancer (NC) causes, while 104,979 (65.8%) were still alive at the end of the follow-up period. Excluding the first year after diagnosis, women with a prior BC diagnosis had a higher risk of mortality (SMR = 328.6.95% CI = 324.8–332.3, p < 0.001) than the age matched female population. NBC mortality risk was elevated for OC (SMR = 103.7, CI = 100.1–107.5, p = 0.022) but reduced for NC causes (SMR = 94.9, CI = 92.3–97.5, p < 0.001). For the age groupings 30–49 years (n = 46,736), 50–69 years (n = 82,967), and 70–79 years (n = 29,847), SMR's for NC causes were 168.2 (CI = 144.1–195.1, p < 0.001), 100.9 (CI = 95.9–106.1, p = 0.355), and 90.5 (CI = 87.5–93.5, p < 0.001) respectively. Following the first year of diagnosis, the SMR for OC was non-significantly elevated with time after diagnosis. In comparison, the SMR for NC causes reduced with time after diagnosis; SMR = 96.4 (CI = 92.1–100.8, p = 0.055) at 2–5 years after diagnosis, 98.2 (CI = 93.7–102.8, p = 0.222) at 6–10 years, 92.8 (CI = 87.2–98.7, p = 0.008) at 11–15 years and 82.2 (CI = 74.8–90.2, p < 0.001) at more than 15 years. Conclusions: BC survivors have a higher risk of mortality from OC compared to the aged-matched Australian female population, but a reduced risk from NC causes. Although NC mortality is lower in older women and as time from BC diagnosis increases, women less than 50 years have a higher risk of NC mortality. No significant financial relationships to disclose.


2016 ◽  
Vol 2016 ◽  
pp. 1-8 ◽  
Author(s):  
Kosuke Hamai ◽  
Hiroshi Iwamoto ◽  
Nobuhisa Ishikawa ◽  
Yasushi Horimasu ◽  
Takeshi Masuda ◽  
...  

Background.Recent reports indicate that matrix metalloproteinase-7 (MMP-7) and CC-chemokine ligand 18 (CCL18) are potential disease markers of idiopathic pulmonary fibrosis (IPF). The objective of this study was to perform direct comparisons of these two biomarkers with three well-investigated serum markers of IPF, Krebs von den Lungen-6 (KL-6), surfactant protein-A (SP-A), and SP-D.Methods.The serum levels of MMP-7, CCL18, KL-6, SP-A, and SP-D were evaluated in 65 patients with IPF, 31 patients with bacterial pneumonia, and 101 healthy controls. The prognostic performance of these five biomarkers was evaluated in patients with IPF.Results.The serum levels of MMP-7, KL-6, and SP-D in patients with IPF were significantly elevated compared to those in patients with bacterial pneumonia and in the healthy controls. Multivariate survival analysis showed that serum MMP-7 and KL-6 levels were independent predictors in IPF patients. Moreover, elevated levels of both KL-6 and MMP-7 were associated with poorer survival rates in IPF patients, and the combination of both markers provided the best risk discrimination using the C statistic.Conclusions.The present results indicated that MMP-7 and KL-6 were promising prognostic markers of IPF, and the combination of the two markers might improve survival prediction in patients with IPF.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Jui-Hui Peng ◽  
Hung-Pin Tu ◽  
Chien-Hui Hong

Abstract Background Tuberous sclerosis complex (TSC) is an autosomal dominant disease with systemic manifestations, which can cause significant mortality and morbidity. Population-based epidemiological studies on TSC mortality and survival remain scarce, though several recent studies provide evidence that TSC survival rates are high and disease prognosis is fair for most patients. This study aims to estimate the life expectancy and mortality statistics in Taiwanese TSC patients, investigate prognosis and associations of TSC mortality based on demographic variables, and compare these results to past literature, especially for Asian patients. Methods Taiwanese National Health Insurance (NHI) insurees can obtain Catastrophic Illness Certificates (CIC) for certain eligible diseases to waive copayments after diagnosis by two independent physicians. CIC holders for TSC during 1997–2010 were identified from the NHI Research Database. Queries on enrollment (CIC acquisition) age, endpoint (end of query period or death) age, sex, and comorbidities were obtained. Patients were separated into cohorts (endpoint age, sex, and age of diagnosis), and analyzed accordingly. Results 471 patients (232 male, 239 female) were identified, of which 14 died. Compared to literature, patients showed similar demographics (age range, diagnosis age, sex distribution); similar manifestations and prevalence (epilepsy, intellectual disability, renal disease); lower disease prevalence (1 in 63,290); lower mortality (0.21% per year); and near-identical standardized mortality ratio (4.99). A cumulative mortality of 4.08% was found over 14 years, though mortality plateaued at 7 years post-enrollment, suggesting a good overall survival rate; comparable with previous studies in Asian patients. Enrollment age was a significant prognostic factor, with late-enrollment (age > 18) patients at higher risk for all-cause mortality (Hazard ratio = 6.54). Average remaining lifetime was significantly lower than the general population, and decreased with age. Conclusions This study reports a population-based disease database, highlights the importance of diagnosis age in prognosis prediction, and suggests the role of renal manifestations in mortality. Furthermore, it corroborates recent TSC studies in the Asian population in terms of survival. Overall, physician vigilance, early diagnosis, and careful monitoring are beneficial for disease outcome and patient survival.


1997 ◽  
Vol 83 (1) ◽  
pp. 9-16 ◽  
Author(s):  
Franco Berrino ◽  
Andrea Micheli ◽  
Milena Sant ◽  
Riccardo Capocaccia

Since 1990 a concerted action between European population-based cancer registries (the EUROCARE project) has been carried out with the aims of establishing whether there are differences in cancer patient survival in Europe, and the reasons for such differences. Survival differences actually exist for cancer sites for which the stage of disease at diagnosis is the major prognostic factor (such as breast, stomach and colon cancer). However, for most cancer sites, survival increases over time and the survival rates of different countries tend to converge towards higher values. Interpreting survival differences and trends is not an easy task. Longer survival may be achieved by postponing death through better treatment or by anticipating diagnosis. However, an earlier diagnosis may or may not make a treatment more effective in postponing death. The computation of stage-specific or stage-adjusted survival is not sufficient for interpretation of survival differences, because staging procedures change over time and may vary in different hospitals and countries. In addition to an early diagnosis and more effective treatment, a number of factors may bias survival estimates. They may be classified into factors that can be controlled in the analysis (at least partially), such as mortality from other causes, demographic factors, epoch of diagnosis, different statistical methodology, and factors depending on the validity of cancer registry data, such as definition of the illness, exhaustiveness and quality of registration, completeness of follow-up, definition of the date of diagnosis, and definition of disease stage including the diagnostic procedure used to establish stage. To help disentangle the effects of early diagnosis and better treatment, several statistical approaches are being developed: multivariate analysis on relative survival data, new modeling analysis to separately estimate the proportion of cured patients and the length of survival for those patients destined to die, and the standardized collection of information on stage at diagnosis and staging procedures.


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