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Respiratory Medicine Jan 2022Pleuroparenchymal fibroelastosis (PPFE) is a rare, generally idiopathic form of interstitial pneumonia with unique clinical, radiological and histopathological features.... (Review)
Review
Pleuroparenchymal fibroelastosis (PPFE) is a rare, generally idiopathic form of interstitial pneumonia with unique clinical, radiological and histopathological features. It is named after the presence of upper lobe pleural and subjacent parenchymal fibrosis, with accompanying elastic fibers. Although it is usually an idiopathic disease, it has been linked to other co-existent diseases. Diagnostic suspicion of PPFE is based on the identification of typical abnormalities on chest CT scan, which are prevailingly located in the upper lobes, adjacent to the apex of the lungs. Diagnosis can be confirmed by histological analysis, although biopsy is not always feasible. The disease is generally progressive, but not uniformly. The course of the disease is frequently slow and involves a progressive loss of upper lobe volume, which results in platythorax, associated with a significant reduction of body mass. PPFE concomitant to other interstitial lung diseases is associated with a poorer prognosis. The disease occasionally progresses rapidly causing irreversible respiratory insufficiency, which leads to death. Currently, there is no effective pharmacological therapy available, and lung transplantation is the best therapeutic option. The purpose of this review is to draw the attention to PPFE, describe its clinical, radiological and histopathological features, analyze its diagnostic criteria, and provide an update on the management of the disease.
Topics: Humans; Lung; Lung Diseases, Interstitial; Lung Transplantation; Pleura; Tomography, X-Ray Computed
PubMed: 33992495
DOI: 10.1016/j.rmed.2021.106437 -
Medicina (Kaunas, Lithuania) Oct 2021: Idiopathic pulmonary fibrosis (IPF) has a variable clinical course, which ranges from being asymptomatic to progressive respiratory failure. The purpose of this study...
: Idiopathic pulmonary fibrosis (IPF) has a variable clinical course, which ranges from being asymptomatic to progressive respiratory failure. The purpose of this study was to evaluate the novel clinical parameters of IPF patients who receive an anti-fibrotic agent. : From January 2011 to January 2021, we identified 39 IPF patients at Okinawa Chubu Hospital. Clinical information was obtained, such as laboratory data, pulmonary function test (PFT) results, and chest images, including of soft tissue thickness and the high-resolution computed tomography (HRCT) pattern at diagnosis. The mean age was 72.9 ± 7.0 (53-85); 27 patients were men and 12 were women. The mean body mass index was 25.1 ± 3.9 (17.3-35). Twenty-four were active smokers and the median number of packs per year was 20. Regarding laboratory findings, mean white blood cell (WBC), lactate dehydrogenase (LDH), and Krebs Von den Lungen-6 (KL-6) values were 7816 ± 1859, 248 ± 47, and 1615 ± 1503, respectively. In PFT, the mean percent predicted FVC, percent predicted total lung capacity, percent predicted functional residual capacity (FRC), and percent predicted diffusion capacity of the lung for carbon monoxide (DLco) were 66.8 ± 14.9%, 71.8 ± 13.7%, 65 ± 39.6%, and 64.6 ± 27.9%, respectively. In chest radiological findings, soft tissue thickness at the right 9th rib was 26.4 ± 8.8 mm. Regarding chest HRCT patterns, 15 showed the definite usual interstitial pneumonia (UIP) pattern, 16 showed the probable UIP pattern, and eight showed the indeterminate for UIP pattern. In the treatment, 24 patients received pirfenidone and 15 patients took nintedanib. The mean observation period was 38.6 ± 30.6 months and 24 patients died. The median survival time was 32.4 months (0.9-142.5). Multivariate analysis adjusted for age showed that both soft tissue thickness [Hazard ratio (HR): 0.912, 95% confidence interval (CI): 0.859-0.979, -value: 0.009] and percent FRC [HR: 0.980, 95% CI: 0.967-0.992, -value: 0.002] were robust predictors of IPF mortality. In IPF patients treated with anti-fibrotic agents, both soft tissue thickness at the right 9th rib shown on the chest radiograph and %FRC can be novel predictors of IPF mortality.
Topics: Aged; Female; Humans; Idiopathic Pulmonary Fibrosis; Lung; Male; Proportional Hazards Models; Respiratory Function Tests; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 34684158
DOI: 10.3390/medicina57101121 -
American Journal of Respiratory Cell... Jul 2022Immune cells have been implicated in idiopathic pulmonary fibrosis (IPF), but the phenotypes and effector mechanisms of these cells remain incompletely characterized. We...
Immune cells have been implicated in idiopathic pulmonary fibrosis (IPF), but the phenotypes and effector mechanisms of these cells remain incompletely characterized. We performed mass cytometry to quantify immune cell subsets in lungs of 12 patients with IPF and 15 organ donors without chronic lung disease and used existing single-cell RNA-sequencing data to investigate transcriptional profiles of immune cells overrepresented in IPF. Among myeloid cells, we found increased numbers of alveolar macrophages (AMØs) and dendritic cells (DCs) in IPF, as well as a subset of monocyte-derived DCs. In contrast, monocyte-like cells and interstitial macrophages were reduced in IPF. Transcriptomic profiling identified an enrichment for IFN-γ response pathways in AMØs and DCs from IPF, as well as antigen processing in DCs and phagocytosis in AMØs. Among T cells, we identified three subsets of memory T cells that were increased in IPF, including CD4 and CD8 resident memory T cells (T) and CD8 effector memory cells. The response to the IFN-γ pathway was enriched in CD4 T and CD8 T cells in IPF, together with T cell activation and immune response-regulating signaling pathways. Increased AMØs, DCs, and memory T cells were present in IPF lungs compared with control subjects. In IPF, these cells possess an activation profile indicating increased IFN-γ signaling and upregulation of adaptive immunity in the lungs. Together, these studies highlight critical features of the immunopathogenesis of IPF.
Topics: Gene Expression Profiling; Humans; Idiopathic Pulmonary Fibrosis; Lung; Macrophages, Alveolar; Single-Cell Analysis
PubMed: 35468042
DOI: 10.1165/rcmb.2021-0418OC -
International Journal of Molecular... Aug 2023Lung fibrosis is a progressive fatal disease in which deregulated wound healing of lung epithelial cells drives progressive fibrotic changes. Persistent lung injury due... (Review)
Review
Lung fibrosis is a progressive fatal disease in which deregulated wound healing of lung epithelial cells drives progressive fibrotic changes. Persistent lung injury due to oxidative stress and chronic inflammation are central features of lung fibrosis. Chronic cigarette smoking causes oxidative stress and is a major risk factor for lung fibrosis. The objective of this manuscript is to develop an adverse outcome pathway (AOP) that serves as a framework for investigation of the mechanisms of lung fibrosis due to lung injury caused by inhaled toxicants, including cigarette smoke. Based on the weight of evidence, oxidative stress is proposed as a molecular initiating event (MIE) which leads to increased secretion of proinflammatory and profibrotic mediators (key event 1 (KE1)). At the cellular level, these proinflammatory signals induce the recruitment of inflammatory cells (KE2), which in turn, increase fibroblast proliferation and myofibroblast differentiation (KE3). At the tissue level, an increase in extracellular matrix deposition (KE4) subsequently culminates in lung fibrosis, the adverse outcome. We have also defined a new KE relationship between the MIE and KE3. This AOP provides a mechanistic platform to understand and evaluate how persistent oxidative stress from lung injury may develop into lung fibrosis.
Topics: Humans; Pulmonary Fibrosis; Adverse Outcome Pathways; Lung Injury; Lung; Oxidative Stress; Fibrosis
PubMed: 37569865
DOI: 10.3390/ijms241512490 -
American Journal of Physiology. Heart... Apr 2021After more than a decade of electronic cigarette (E-cig) use in the United States, uncertainty persists regarding E-cig use and long-term cardiopulmonary disease risk....
After more than a decade of electronic cigarette (E-cig) use in the United States, uncertainty persists regarding E-cig use and long-term cardiopulmonary disease risk. As all E-cigs use propylene glycol and vegetable glycerin (PG-VG) and generate abundant saturated aldehydes, mice were exposed by inhalation to PG-VG-derived aerosol, formaldehyde (FA), acetaldehyde (AA), or filtered air. Biomarkers of exposure and cardiopulmonary injury were monitored by mass spectrometry (urine metabolites), radiotelemetry (respiratory reflexes), isometric myography (aorta), and flow cytometry (blood markers). Acute PG-VG exposure significantly affected multiple biomarkers including pulmonary reflex (decreased respiratory rate, -50%), endothelium-dependent relaxation (-61.8 ± 4.2%), decreased WBC (-47 ± 7%), and, increased RBC (+6 ± 1%) and hemoglobin (+4 ± 1%) versus air control group. Notably, FA exposure recapitulated the prominent effects of PG-VG aerosol on pulmonary irritant reflex and endothelial dysfunction, whereas AA exposure did not. To attempt to link PG-VG exposure with FA or AA exposure, urinary formate and acetate levels were measured by GC-MS. Although neither FA nor AA exposure altered excretion of their primary metabolite, formate or acetate, respectively, compared with air-exposed controls, PG-VG aerosol exposure significantly increased post-exposure urinary acetate but not formate. These data suggest that E-cig use may increase cardiopulmonary disease risk independent of the presence of nicotine and/or flavorings. This study indicates that FA levels in tobacco product-derived aerosols should be regulated to levels that do not induce biomarkers of cardiopulmonary harm. There remains a need for reliable biomarkers of exposure to inhaled FA and AA. Use of electronic cigarettes (E-cig) induces endothelial dysfunction (ED) in healthy humans, yet the specific constituents in E-cig aerosols that contribute to ED are unknown. Our study implicates formaldehyde that is formed in heating of E-cig solvents (propylene glycol, PG; vegetable glycerin, VG). Exposure to formaldehyde or PG-VG-derived aerosol alone stimulated ED in female mice. As ED was independent of nicotine and flavorants, these data reflect a "universal flaw" of E-cigs that use PG-VG.Listen to this article's corresponding podcast at https://ajpheart.podbean.com/e/e-cigarettes-aldehydes-and-endothelial-dysfunction/.
Topics: Acetaldehyde; Aerosols; Animals; Aorta, Thoracic; Biomarkers; E-Cigarette Vapor; Endothelium, Vascular; Female; Formaldehyde; Glycerol; Inhalation Exposure; Lung; Male; Mice, Inbred C57BL; Propylene Glycol; Respiration; Risk Assessment; Solvents; Vasoconstriction; Vasodilation; Mice
PubMed: 33543686
DOI: 10.1152/ajpheart.00878.2020 -
The Journal of Allergy and Clinical... Dec 2022In addition to being a vital organ for gas exchange, the lung is a crucial immune organ continuously exposed to the external environment. Genetic defects that impair... (Review)
Review
In addition to being a vital organ for gas exchange, the lung is a crucial immune organ continuously exposed to the external environment. Genetic defects that impair immune function, called inborn errors of immunity (IEI), often have lung disease as the initial and/or primary manifestation. Common types of lung disease seen in IEI include infectious complications and a diverse group of diffuse interstitial lung diseases. Although lung damage in IEI has been historically ascribed to recurrent infections, contributions from potentially targetable autoimmune and inflammatory pathways are now increasingly recognized. This article provides a practical guide to identifying the diverse pulmonary disease patterns in IEI based on lung imaging and respiratory manifestations, and integrates this clinical information with molecular mechanisms of disease and diagnostic assessments in IEI. We cover the entire IEI spectrum, including immunodeficiencies and immune dysregulation with monogenic autoimmunity and autoinflammation, as well as recently described IEI with pulmonary manifestations. Although the pulmonary manifestations of IEI are highly relevant for all age groups, special emphasis is placed on the pediatric population, because initial presentations often occur during childhood. We also highlight the pivotal role of genetic testing in the diagnosis of IEI involving the lungs and the critical need to develop multidisciplinary teams for the challenging evaluation of these rare but potentially life-threatening disorders.
Topics: Child; Humans; Autoimmunity; Genetic Testing; Lung; Lung Diseases
PubMed: 36244852
DOI: 10.1016/j.jaci.2022.08.024 -
Respiratory Care Apr 2021The purposes of this study were to verify the correlation between chest expansion and lung function within a larger sample of subjects composed of both healthy subjects...
BACKGROUND
The purposes of this study were to verify the correlation between chest expansion and lung function within a larger sample of subjects composed of both healthy subjects and subjects affected by pulmonary disease, and to verify the influence of age, body mass index, and gender on chest expansion.
METHODS
Adults were recruited prospectively when they visited the lung function lab. Chest expansion was measured with a measuring tape at 2 different levels of the rib cage by 1 blinded examiner. Spirometry was performed for each subject.
RESULTS
Data from 251 subjects between 18 and 88 y old were collected and analyzed. Among the analyzed subjects, mean upper and lower chest expansion were 4.82 ± 1.84 cm and 3.99 ± 2.15 cm, respectively. A significant but poor correlation was found between both chest expansion and all lung function parameters (total lung capacity, FVC, and FEV) ( = .01). Negative significant correlations were found between chest expansion and age as well as body mass index. The difference in upper chest expansion between obese and nonobese subjects was not statistically significant, but the difference in lower chest expansion was significant for these 2 groups. Finally, upper and lower chest expansion were not different between males and females.
CONCLUSIONS
Based on these results, one cannot validate the use of chest expansion measurement to define lung function. In centers that have easy access to more precise and complete methods to measure lung function, the measurement of chest expansion for diagnostic purposes seems to be archaic. Additionally, age and body mass index are 2 parameters that can influence chest expansion.
Topics: Adult; Female; Healthy Volunteers; Humans; Lung; Lung Diseases; Male; Respiratory Muscles; Thoracic Wall
PubMed: 33376188
DOI: 10.4187/respcare.08350 -
Ghana Medical Journal Jun 2020Pulmonary tuberculosis manifesting as a mass lesion, thus, mimicking a lung carcinoma is an unusual radiographic presentation of tuberculosis (TB). The common radiologic...
UNLABELLED
Pulmonary tuberculosis manifesting as a mass lesion, thus, mimicking a lung carcinoma is an unusual radiographic presentation of tuberculosis (TB). The common radiologic patterns and clinical presentations are well known and documented. We report two cases of pulmonary tuberculosis with a neoplastic appearance on chest imaging diagnosed histologically. A 21 - year old female with cough, weight loss, anorexia and an unremarkable physical examination. Chest radiography showed a right apical mass suggestive of lung cancer. Histology of the lesion revealed parenchymal pulmonary tuberculosis. A 49-year old male with left-sided chest pain, cough, anorexia, weight loss, mild pallor with an unremarkable chest examination. Chest imaging showed a left apical mass and mediastinal lymphadenopathy. Microscopic examination of the mass confirmed pulmonary tuberculosis. Pseudotumour pulmonary tuberculosis is a rare clinical entity that can lead to diagnostic challenges and must be considered in the differential diagnosis when mass lesions are seen on chest imaging, especially in TB endemic areas.
FUNDING
None declared.
Topics: Antitubercular Agents; Female; Humans; Lung; Male; Middle Aged; Radiography; Tomography, X-Ray Computed; Treatment Outcome; Tuberculosis, Pulmonary; Young Adult
PubMed: 33536684
DOI: 10.4314/gmj.v54i2.12 -
Aging Dec 2023Aging usually causes lung-function decline and susceptibility to chronic lung diseases, such as pulmonary fibrosis. However, how aging affects the lung-fibrosis pathways...
Aging usually causes lung-function decline and susceptibility to chronic lung diseases, such as pulmonary fibrosis. However, how aging affects the lung-fibrosis pathways and leads to the occurrence of pulmonary fibrosis is not completely understood. Here, mass spectrometry-based proteomics was used to chart the lung proteome of young and old mice. Micro computed tomography imaging, RNA immunoprecipitation, dual-fluorescence mRFP-GFP-LC3 adenovirus monitoring, transmission electron microscopy, and other experiments were performed to explore the screened differentially expressed proteins related to abnormal ferroptosis, autophagy, mitochondria, and mechanical force , , and in healthy people. Combined with our previous studies on pulmonary fibrosis, we further demonstrated that these biological processes and underlying molecular players were also involved in the aging process. Our work depicted a comprehensive cellular and molecular atlas of the aging lung and attempted to explain why aging is a risk factor for pulmonary fibrosis and the role that aging plays in the progression of pulmonary fibrosis. The abnormalities of aging triggered an increase in mechanical force and ferroptosis, autophagy blockade, and mitochondrial dysfunction, which often appear during pulmonary fibrogenesis. We hope that the elucidation of these anomalies will help to enhance our understanding of senescence-inducing pulmonary fibrosis, thereby guiding the use of anti-senescence as an entry point for early intervention in pulmonary fibrosis and age-related diseases.
Topics: Humans; Animals; Mice; Proteomics; X-Ray Microtomography; Idiopathic Pulmonary Fibrosis; Lung; Aging; MicroRNAs; Cellular Senescence
PubMed: 38147026
DOI: 10.18632/aging.205355 -
Nutrients Sep 2022Background: Given the potential risk of unhealthy weight management, the monitoring of body composition in athletes is advised. However, limited data reveal how body...
Background: Given the potential risk of unhealthy weight management, the monitoring of body composition in athletes is advised. However, limited data reveal how body composition measurements can benefit athlete health and, in particular, respiratory function. The aim of this study is to evaluate the impact of body composition on pulmonary function in a population of adult athletes. Methods: Data from 435 competitive adult athletes regarding body compositions parameters and spirometry are retrospectively analyzed. Results: Our study population consists of 335 males and 100 female athletes. Muscle mass and fat-free mass are significantly and positively associated with forced expiratory volume in the first second (FEV1) and forced vital capacity (FVC) in the male and female population, while waist-to-height ratio is negatively associated with FEV1, FVC, and FEV1/FVC in the male population. In multivariable analysis, muscle mass and fat-free mass show significant association with FEV1 and FVC in both males and females (p < 0.05), and waist-to-height ratio is significantly and inversely associated with FEV1 and FVC in males (p < 0.05). Conclusions: Fat-free mass and muscle mass are positively and independently associated with FEV1 and FVC in athletes of both genders, and waist-to-height ratio is inversely associated with FEV1 and FVC only among male athletes. These findings suggest that body composition in athletes may be helpful in monitoring respiratory function.
Topics: Adult; Athletes; Body Composition; Body Mass Index; Female; Forced Expiratory Volume; Humans; Lung; Male; Retrospective Studies; Spirometry; Vital Capacity
PubMed: 36145219
DOI: 10.3390/nu14183844