@article{kaunisto2019demographics,
title={Demographics and survival of patients with idiopathic pulmonary fibrosis in the FinnishIPF registry},
author={Kaunisto, Jaana and Salomaa, Eija-Riitta and Hodgson, Ulla and Kaarteenaho, Riitta and Kankaanranta, Hannu and Koli, Katri and Vahlberg, Tero and Myll{\"a}rniemi, Marjukka},
journal={ERJ open research},
volume={5},
number={3},
pages={00170--2018},
year={2019},
publisher={Eur Respiratory Soc}
}
FinnishIPF, a nationwide registry of carefully characterised patients, was initiated in Finland in 2011. For the data analysis, we included 453 incident IPF patients diagnosed during 2011–2015. In this study, we describe the demographics and prognosis of these real-life patients.
The median overall survival time of registered IPF patients was 4.5 years. The transplant-free survival at 1, 2, 3, 4 and 5 years was 95%, 83%, 70%, 58% and 45%, respectively. Smoking did not have any effect on survival. 117 (26%) patients received pirfenidone or nintedanib. Patients who received ≥6 months of treatment had better survival compared with those who did not receive treatment but this difference disappeared after age adjustment. The transplantation rate was 3%.
Although IPF is diagnosed in Finland at a older age, the prognosis is better than expected due to a relatively well preserved lung function at diagnosis. Age and pulmonary function were identified as independent predictors of survival in the entire IPF patient population as well as in patients who had received antifibrotic treatment.
@article{cottin2017burden,
title={Burden of idiopathic pulmonary fibrosis progression: a 5-year longitudinal follow-up study},
author={Cottin, Vincent and Schmidt, Aur{\'e}lie and Catella, Laura and Porte, Fanny and Fernandez-Montoya, C{\'e}line and Le Lay, Katell and B{\'e}nard, St{\`e}ve},
journal={PLoS One},
volume={12},
number={1},
pages={e0166462},
year={2017},
publisher={Public Library of Science San Francisco, CA USA}
}J84.1:
The ICD code J841 is used to code Acute interstitial pneumonitis Acute interstitial pneumonitis (also known as acute interstitial pneumonia or Hamman–Rich syndrome) is a rare, severe lung disease that usually affects otherwise healthy individuals. There is no known cause or cure.
J84.10 Pulmonary fibrosis, unspecified (516.31)
J84.11 Idiopathic interstitial pneumonia (516.3)
J84.17 Other interstitial pulmonary diseases with fibrosis in diseases classified elsewhere (516.3)
Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause and limited to the lungs. It is a rare disease with an incidence estimated at 3.81 per 100,000 inhabitants in Europe [1]. According to an international consensus statement jointly issued by the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society and the Latin American Thoracic Association, IPF is a distinct clinical entity associated with the histological and/or radiological appearance of usual interstitial pneumonia [2]. The definition of IPF requires the exclusion of other forms of interstitial pneumonia including other idiopathic interstitial pneumonias and interstitial lung diseases associated with environmental exposure, medication or systemic disease. IPF predominantly presents in individuals older than 50 years [2,3] with a preponderance in men and previous or current smokers. Patients present with chronic exertional dyspnoea, bibasilar inspiratory crackles, and commonly with cough, and finger clubbing. IPF is characterised by progressive worsening of dyspnoea and lung function. This chronic disease is associated with a poor prognosis [2,4]. The median survival of patients diagnosed with this condition is estimated between 3 and 5 years [5]. The natural disease course is variable and characterised by various acute events, of whom idiopathic acute exacerbations are a major concern [6,7]. Currently there are no markers to reliably predict the clinical course in an individual patient. Little is known about the incidence and outcomes after acute events in Europe. While studies with small samples have focused on the poor prognosis of patients with severe IPF admitted to intensive care units in Europe and in the United States (US) [8–12], only one recent single-centre US study has evaluated the reasons for hospital admission and the outcomes for patients with IPF [13]. The results showed that hospitalisations, especially those related to respiratory problems, were common, and were associated with high in-hospital mortality.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive-fibrosing interstitial lung disease of unknown origin that affects 3 million people worldwide and imparts substantial burdens to patients, their families, and the healthcare system. The IPF disease course is highly variable and presents several diagnostic and management-related challenges.
@article{glassberg2019overview,
title={Overview of idiopathic pulmonary fibrosis, evidence-based guidelines, and recent developments in the treatment landscape},
author={Glassberg, Marilyn K},
journal={Am J Manag Care},
volume={25},
pages={S195--203},
year={2019}
}Overview of Idiopathic Pulmonary Fibrosis and Evidence-Based Guidelines Am J Manag Care. 2019;25:-S0
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive-fibrosing interstitial lung disease (ILD) of unknown origin characterized by progressive lung scarring and the histologic picture of usual interstitial pneumonia.1,2 Disorders belonging to the ILD category cause damage to the lung interstitium through various mechanisms, including inflammation, edema, and/or fibrosis.3 Despite sharing common clinical and pathophysiologic features, ILDs are a group of heterogenous diseases with diverse etiologies and prognoses.4 Accounting for 55% of all ILDs, IPF results in dilation of the bronchi, alveolar remodeling, and bibasilar parenchymal fibrosis, all of which contribute to scarring that leads to impaired gas exchange, particularly oxygenation, as shown in Figure 1.5
The disease course of IPF is highly variable; most patients progress more slowly while others experience rapid lung decline.6 In addition, patients with IPF may have periods of relatively stable disease interspersed with acute deteriorations in lung function.7 Therefore, the clinical course of an individual patient is difficult to predict; however, the median survival for patients with IPF before the era of antifibrotics has been 3 to 5 years following diagnosis.8
The incidence of IPF increases with age, and diagnosis before age 50 is rare.9,10 Among adults aged 18 to 64 years, the annual incidence is approximately 6 cases per 100,000 person-years,11 yet in adults 65 years and older, this incidence climbs to 94 cases per 100,000 person-years.12 Similarly, the prevalence of IPF is 18 cases per 100,000 adults aged 18 to 64 years,11 whereas in individuals 65 years and older, the prevalence is 495 cases per 100,000.12 Incidence and prevalence data vary widely due to fundamental differences in data collection methods and definitions of IPF. Collectively, it has been estimated that 130,000 people in the United States have been diagnosed with IPF.3 A claims analysis of US Medicare beneficiaries from 2000 to 2011 found that older age and male sex were significantly associated with a higher incidence of IPF and shorter survival time following diagnosis.12 In addition, thyroid disease, diabetes, coronary artery disease (CAD), and lung cancer have been associated with shorter survival in patients with IPF.13,14
Along with increasing age and male sex, potential risk factors for IPF include cigarette smoking, environmental exposures, microbial pathogens, and genetic factors.1 Anyone with a smoking history has a 60% higher risk of developing IPF.15 A retrospective analysis found that current smokers are 13 to 14 years younger at diagnosis compared with nonsmokers and former smokers (58.1, 71.4, and 72.5 years, respectively).13 Occupational exposures that may contribute to IPF include agriculture and farming; livestock; silica; and wood, metal, or stone dust.16 Some viruses, such as the Epstein-Barr virus, may also play a part in IPF development.3 The lung microbiota has a much higher bacterial load in patients with IPF than those without; understanding the possible role of bacteria in IPF pathogenesis is the focus of the current trial Clean-UP IPF for the Pulmonary Clinical Trials Cooperative (NCT02759120).17
A cohort study by Adegunsoye et al investigated survival rates in African American patients diagnosed with IPF. Despite being diagnosed at an earlier age, having poor measures of lung function and similar rates of hospitalizations as the cohort population, African Americans exhibited longer survival times. These findings suggest that race and genetics may play a role in the survival advantage exhibited by African American patients.18 A recent study has identified American Indians/Alaska Natives as the racial group with the highest IPF-related mortality rate.19 The interracial differences observed may support a genetic basis for predisposition to disease. Other factors to explore are whether behavioral or environmental risk factors differ by race. For instance, it is known that smoking rates differ by race, with the highest rates observed among American Indians.20
Comorbidities are substantial among patients with IPF and include hypertension, pulmonary hypertension (PH), obstructive sleep apnea, lung cancer, chronic obstructive pulmonary disease, CAD, vascular disease, diabetes, and gastroesophageal reflux disease (GERD).13 Effective management of comorbidities contributes to survival and may positively impact the IPF disease course.21 Micro-aspirations of gastric content may be involved in the lung injury leading to IPF3; however, the relationship between GERD and IPF may be confounded by smoking. A recent meta-analysis reported that a significant association between GERD and IPF (odds ratio, 2.94; P <.0001) was found when 18 case-control studies (3206 cases of IPF and 9368 controls) were pooled, but this association disappeared when investigators controlled for smoking status.22
The pathogenesis of IPF is multifactorial and involves the convergence of 3 elements: (1) epithelial damage, (2) lung tissue destruction, and (3) accelerated aging-associated changes.3 The combination of these elements leads to the release of mediators that induce migration, proliferation, and activation of fibroblasts and myofibroblasts that resist apoptosis and secrete extracellular matrix. Growth factors are released that contribute to the relentless progression of the disease.3
The hallmark clinical signs of IPF are nonproductive cough and progressive exertional dyspnea, and approximately one-third of patients with IPF will have digital clubbing. Scalene muscle hypertrophy and bibasilar fine crackles should also raise suspicion of IPF.23 The diagnosis of IPF in suspected cases involves an in-depth review of both medication and environmental exposure histories followed by a high-resolution computed tomography scan (HRCT). Depending on the results of the HRCT, an analysis of the bronchoalveolar lavage fluid or surgical lung biopsy may be performed.8 An evaluation of the HRCT results combined with a histopathology pattern confirms a diagnosis of IPF.8 Other conditions, such as systemic sclerosis ILD (SSc-ILD, a type of connective tissue disease [CTD]) and rheumatoid arthritis-associated ILD (RA-ILD), have a similar pathophysiology as IPF and should be considered in a differential diagnosis.23 Findings that are suggestive of an alternative diagnosis include pleural plaques (consider asbestosis), dilated esophagus (consider CTD including SSc), distal clavicular erosions (consider RA), extensive lymph node enlargement (consider other etiologies), and pleural effusions and/or thickening (consider CTD/drugs).8
As a result of overlapping comorbidities and lack of specific symptoms, delays in the diagnosis of IPF are common. Lamas et al found a median delay of 2.2 years (interquartile range [IQR], 1.1-3.8 years) between the onset of dyspnea and the date of the initial evaluation for IPF. As shown in Figure 2,24 a longer delay was associated with shortened survival following initial evaluation (hazard ratio [HR], 1.3; 95% CI, 1.03-1.6; P = .03). Patients who waited 4 years or longer for a diagnosis had higher rates of CAD, diabetes, and GERD at baseline.24 Therefore, any efforts to improve the early recognition and diagnosis of patients with IPF can greatly impact outcomes.