scholarly journals Global incidence and prevalence of idiopathic pulmonary fibrosis

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Toby M. Maher ◽  
Elisabeth Bendstrup ◽  
Louis Dron ◽  
Jonathan Langley ◽  
Gerald Smith ◽  
...  
Keyword(s):  
North America ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
South Korea ◽  
Rare Disease ◽  
Epidemiologic Studies ◽  
Asia Pacific ◽  
Prevalence Estimates ◽  
Definition Of ◽  
Adjusted Incidence

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.

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
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Cause Of Death ◽  
Normal Population ◽  
Survival Rates ◽  
Population Based ◽  
Epidemiologic Studies ◽  
Standardized Mortality Ratio ◽  
Mortality Ratio ◽  
Definition Of

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.

Idiopathic pulmonary fibrosis: a more common condition than you may think

2021 ◽  
Vol 30 (6) ◽  
pp. 359-366
Author(s):  
Penny Tremayne ◽  
Samuel John Clark
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Health Professionals ◽  
Treatment Options ◽  
Delayed Diagnosis ◽  
Common Condition ◽  
Inadequate Care ◽  
Definition Of ◽  
The Uk ◽  
Causes And Risk Factors

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive incurable lung disease that affects a significant amount of people in the UK. Many health professionals have a limited understanding of IPF, which can result in a delayed diagnosis and inadequate care for individuals and their families. This article aims to provide an overview of IPF and help to enhance health professionals' understanding of the disease, thus contributing towards improving the care that IPF sufferers receive. This article provides a definition of IPF and explores its pathophysiology. It discusses the causes and risk factors for developing the condition, examines how IPF is diagnosed and details the treatment options available for IPF patients.

The definition of fibrogenic processes in fibroblastic foci of idiopathic pulmonary fibrosis based on morphometric quantification of extracellular matrices

2009 ◽  
Vol 40 (9) ◽  
pp. 1278-1287 ◽  
Author(s):  
Masahiro Yamashita ◽  
Kohei Yamauchi ◽  
Ryoji Chiba ◽  
Noriyuki Iwama ◽  
Fumiko Date ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Extracellular Matrices ◽  
Definition Of

Idiopathic pulmonary fibrosis a rare disease with severe bone fragility

2016 ◽  
Vol 11 (8) ◽  
pp. 1087-1094 ◽  
Author(s):  
Carla Caffarelli ◽  
Stefano Gonnelli ◽  
Maria Dea Tomai Pitinca ◽  
Valentina Francolini ◽  
Annalisa Fui ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Rare Disease ◽  
Bone Fragility

scholarly journals Boehringer Ingelheim Summer 2019 Poster Presentation/Clinical Research Data

2020 ◽  
Author(s):  
Giridhar Murali
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Clinical Research ◽  
Poster Presentation ◽  
Rare Disease ◽  
Patient Experience ◽  
Idiopathic Pulmonary Fibrosis Patient ◽  
Research Data ◽  
Clinical Research Data

This poster presentation contains clinical research data that was used in order to enhance IPF (Idiopathic Pulmonary Fibrosis) patient experience, and education about the OFEV drug to treat the rare disease. This data and presentation was published internally in Boehringer Ingelheim.

scholarly journals Idiopathic pulmonary fibrosis: prognostic impact of histologic honeycombing in transbronchial lung cryobiopsy

2019 ◽  
Vol 14 ◽  
Author(s):  
Claudia Ravaglia ◽  
Marcello Bosi ◽  
Athol U. Wells ◽  
Carlo Gurioli ◽  
Christian Gurioli ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Usual Interstitial Pneumonia ◽  
Prognostic Impact ◽  
Surgical Biopsy ◽  
Free Survival ◽  
Risk Of Death ◽  
Transbronchial Lung Cryobiopsy ◽  
Definition Of

Background: Prognostic evaluation in idiopathic pulmonary fibrosis (IPF) may be important as it can guide management decisions, but the potential role of honeycomb changes in providing information about outcome and survival of patients with IPF, particularly if diagnosed using cryobiopsy, has not been evaluated. Aim of this study was to determinate whether a relationship exists between honeycombing on cryobiopsy and clinical/radiological picture and outcome in patients with IPF and to assess whether the same pathologic criteria that have been used to define the UIP pattern (usual interstitial pneumonia) for surgical biopsy can also be applied to cryobiopsy. Methods: Sixty-three subjects with a multidisciplinary diagnosis of IPF and a UIP pattern on cryobiopsy were evaluated. Patients were classified into two sub-groups depending on the presence of honeycombing on histology. Results: The presence of honeycombing on cryobiopsy did not identify a specific phenotype of patients as it did not correlate with radiological and clinical picture and it was not associated neither with the risk of death (p = 0. 1192) or with the event-free survival (p = 0.827); a higher number of samples and the presence of pleura on biopsy were instead associated with an increase in the finding of honeycombing. Conclusions: The same pathologic criteria that have been used to define the UIP pattern in surgical biopsies (with honeycombing changes considered as non-mandatory for the definition of the pattern itself) can be applied to cryobiopsy samples, as the presence of these changes do not define different clinical or radiological phenotypes of patients with IPF.

Sign in / Sign up

Export Citation Format

Share Document