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The European Respiratory Journal Jan 2020The prevailing view is that exertional dyspnoea in patients with combined idiopathic pulmonary fibrosis (IPF) and emphysema (CPFE) can be largely explained by severe...
The prevailing view is that exertional dyspnoea in patients with combined idiopathic pulmonary fibrosis (IPF) and emphysema (CPFE) can be largely explained by severe hypoxaemia. However, there is little evidence to support these assumptions.We prospectively contrasted the sensory and physiological responses to exercise in 42 CPFE and 16 IPF patients matched by the severity of exertional hypoxaemia. Emphysema and pulmonary fibrosis were quantified using computed tomography. Inspiratory constraints were assessed in a constant work rate test: capillary blood gases were obtained in a subset of patients.CPFE patients had lower exercise capacity despite less extensive fibrosis compared to IPF (p=0.004 and 0.02, respectively). Exertional dyspnoea was the key limiting symptom in 24 CPFE patients who showed significantly lower transfer factor, arterial carbon dioxide tension and ventilatory efficiency (higher minute ventilation (')/carbon dioxide output (' ) ratio) compared to those with less dyspnoea. However, there were no between-group differences in the likelihood of pulmonary hypertension by echocardiography (p=0.44). High dead space/tidal volume ratio, low capillary carbon dioxide tension emphysema severity (including admixed emphysema) and traction bronchiectasis were related to a high '/' ratio in the more dyspnoeic group. '/' nadir >50 (OR 9.43, 95% CI 5.28-13.6; p=0.0001) and total emphysema extent >15% (2.25, 1.28-3.54; p=0.01) predicted a high dyspnoea burden associated with severely reduced exercise capacity in CPFEContrary to current understanding, hypoxaemia is not the main determinant of exertional dyspnoea in CPFE. Poor ventilatory efficiency due to increased "wasted" ventilation in emphysematous areas and hyperventilation holds a key mechanistic role that deserves therapeutic attention.
Topics: Dyspnea; Emphysema; Exercise Test; Exercise Tolerance; Humans; Pulmonary Emphysema
PubMed: 31649067
DOI: 10.1183/13993003.01319-2019 -
The European Respiratory Journal Oct 2001Accurate diagnosis and quantification of pulmonary emphysema during life is important to understand the natural history of the disease, to assess the extent of the... (Review)
Review
Accurate diagnosis and quantification of pulmonary emphysema during life is important to understand the natural history of the disease, to assess the extent of the disease, and to evaluate and follow-up therapeutic interventions. Since pulmonary emphysema is defined through pathological criteria, new methods of diagnosis and quantification should be validated by comparisons against histological references. Recent studies have addressed the capability of computed tomography (CT) to quantify pulmonary emphysema accurately. The studies reviewed in this article have been based on CT scans obtained after deep inspiration or expiration, on subjective visual grading and on objective measurements of attenuation values. Especially dedicated software was used for this purpose, which provided numerical data, on both two- and three-dimensional approaches, and compared CT data with pulmonary function tests. More recently, fractal and textural analyses were applied to computed tomography scans to assess the presence, the extent, and the types of emphysema. Quantitative computed tomography has already been used in patient selection for surgical treatment of pulmonary emphysema and in pharmacotherapeutical trials. However, despite numerous and extensive studies, this technique has not yet been standardized and important questions about how best to use computed tomography for the quantification of pulmonary emphysema are still unsolved.
Topics: Humans; Image Processing, Computer-Assisted; Lung; Pulmonary Emphysema; Respiratory Function Tests; Tomography, X-Ray Computed
PubMed: 11716178
DOI: 10.1183/09031936.01.00255701 -
International Journal of Chronic... 2019Although congenital lobar emphysema is a rare lung disease, it can cause severe respiratory distress in the newborn. Lobectomy can be difficult because of the... (Review)
Review
Although congenital lobar emphysema is a rare lung disease, it can cause severe respiratory distress in the newborn. Lobectomy can be difficult because of the hyperinflated lobe and limited space to carry out surgery. During the past two decades, conservative treatment options have increased for patients with mild and moderate disease.
Topics: Bronchoscopy; Female; Humans; Infant; Infant, Newborn; Lung; Magnetic Resonance Imaging; Male; Pneumonectomy; Predictive Value of Tests; Pulmonary Emphysema; Risk Factors; Tomography, X-Ray Computed; Treatment Outcome; Ultrasonography, Prenatal
PubMed: 31118601
DOI: 10.2147/COPD.S170581 -
The Journal of Clinical Investigation Feb 2008Emphysema is one manifestation of a group of chronic, obstructive, and frequently progressive destructive lung diseases. Cigarette smoking and air pollution are the main... (Review)
Review
Emphysema is one manifestation of a group of chronic, obstructive, and frequently progressive destructive lung diseases. Cigarette smoking and air pollution are the main causes of emphysema in humans, and cigarette smoking causes emphysema in rodents. This review examines the concept of a homeostatically active lung structure maintenance program that, when attacked by proteases and oxidants, leads to the loss of alveolar septal cells and airspace enlargement. Inflammatory and noninflammatory mechanisms of disease pathogenesis, as well as the role of the innate and adaptive immune systems, are being explored in genetically altered animals and in exposure models of this disease. These recent scientific advances support a model whereby alveolar destruction resulting from a coalescence of mechanical forces, such as hyperinflation, and more recently recognized cellular and molecular events, including apoptosis, cellular senescence, and failed lung tissue repair, produces the clinically recognized syndrome of emphysema.
Topics: Animals; Disease Models, Animal; Guinea Pigs; Humans; Immunity, Innate; Mice; Oxidative Stress; Peptide Hydrolases; Pulmonary Emphysema; Rats; alpha 1-Antitrypsin
PubMed: 18246188
DOI: 10.1172/JCI31811 -
Respirology (Carlton, Vic.) Jan 2019Combined pulmonary fibrosis and emphysema (CPFE) is characterized by preserved lung volume and slower lung function decline. However, it is unclear at what extent...
BACKGROUND AND OBJECTIVE
Combined pulmonary fibrosis and emphysema (CPFE) is characterized by preserved lung volume and slower lung function decline. However, it is unclear at what extent emphysema begins to impact respiratory physiology and prognostic characteristics in idiopathic pulmonary fibrosis (IPF). We estimated the extent of emphysema that could be used to define CPFE in IPF.
METHODS
The extent of emphysema was observed on high-resolution computed tomography scans and measured by a texture-based automated quantification system in 209 IPF patients. We analysed the impact of differences in the extent of emphysema on the annual decline rate and prognostic significance of lung function parameters.
RESULTS
The extent of emphysema was ≥5% in 53 patients (25%), ≥10% in 23 patients (11%) and ≥15% in 12 patients (6%). Patients with emphysema to an extent of ≥5% were more frequently men and ever-smokers; they had more preserved lung volume and lower forced vital capacity (FVC) decline rates than those with no or trivial emphysema. The FVC decline rate was a significant predictor of mortality in patients with no or trivial emphysema (hazard ratio (HR): 0.933, P < 0.001) and in patients with an extent of emphysema ≥5% (HR: 0.906, P < 0.001). However, diffusing capacity of the lung for carbon monoxide (DL ) was the most significant prognostic factor in those patients with an extent of emphysema ≥10% (HR: 0.972, P = 0.040) and ≥15% (HR: 0.942, P = 0.023). A 10% cut-off value for the extent of emphysema created the most significant difference in the annual FVC decline rate in IPF patients.
CONCLUSION
In IPF, emphysema to an extent of ≥10% affects both the annual decline rate and the prognostic significance of FVC. This extent could be used to define CPFE.
Topics: Aged; Computing Methodologies; Female; Humans; Idiopathic Pulmonary Fibrosis; Lung; Male; Middle Aged; Prognosis; Proportional Hazards Models; Pulmonary Emphysema; Respiratory Function Tests; Tomography, X-Ray Computed; Vital Capacity
PubMed: 30136753
DOI: 10.1111/resp.13387 -
Proceedings of the American Thoracic... May 2008Chronic obstructive pulmonary disease (COPD) is characterized physiologically by expiratory flow limitation and pathologically by alveolar destruction and enlargement... (Review)
Review
Chronic obstructive pulmonary disease (COPD) is characterized physiologically by expiratory flow limitation and pathologically by alveolar destruction and enlargement and small and large airway inflammation and remodeling. An imbalance between protease and antiprotease activity in the lung is proposed as the major mechanism resulting in emphysema. The imbalance is mostly due to an increase in the numbers of alveolar macrophages and neutrophils. Emphysema can also develop from increased alveolar wall cell death and/or failure in alveolar wall maintenance. Chronic inflammation and increased oxidative stress contribute to increased destruction and/or impaired lung maintenance and repair. Genetic factors may play an important role in disease susceptibility because only a minority of smokers develops emphysema. Recent literature implicates surfactant instability, malnutrition, and alveolar cell apoptosis as possible etiologies. Identification of cellular and molecular mechanisms of COPD pathogenesis is an area of active, ongoing research that may help to determine therapeutic targets for emphysema.
Topics: Animals; Apoptosis; Endopeptidases; Humans; Pulmonary Alveoli; Pulmonary Emphysema; Pulmonary Surfactants; Risk Factors
PubMed: 18453358
DOI: 10.1513/pats.200708-126ET -
Monaldi Archives For Chest Disease =... Dec 2011Chronic obstructive pulmonary disease (that includes emphysema) results in significant morbidity and mortality worldwide. Idiopathic pulmonary fibrosis (IPF) is also a...
Chronic obstructive pulmonary disease (that includes emphysema) results in significant morbidity and mortality worldwide. Idiopathic pulmonary fibrosis (IPF) is also a chronic and progressive parenchymal lung disease with an average survival of less than 5 years after diagnosis. Combined pulmonary fibrosis and emphysema (CPFE) is an important but still underdiagnosed syndrome. Its diagnosis is based on the radiological findings at computed tomography which consists of emphysema of the upper lung zones and fibrosis of the lower lung zones. Since this syndrome has a very bad prognosis, even worse than isolated finding of emphysema or fibrosis alone, early recognition and rapid treatment are important. In this article we will review and elucidate the radiologic appearance of this syndrome and highlight its clinical importance.
Topics: Humans; Prognosis; Pulmonary Emphysema; Pulmonary Fibrosis; Syndrome; Tomography, X-Ray Computed
PubMed: 22462310
DOI: 10.4081/monaldi.2011.210 -
The Surgical Clinics of North America Oct 2010This article addresses several distinct but related pulmonary conditions that are commonly referred to general thoracic surgeons for decision making and management. The... (Review)
Review
This article addresses several distinct but related pulmonary conditions that are commonly referred to general thoracic surgeons for decision making and management. The management of various types of pneumothorax is reviewed, with particular attention to the selection of the appropriate level of surgical intervention. The related entities of bullous lung disease and diffuse emphysema are discussed, with a focus on the identification of appropriate circumstances for surgical intervention. The summarized work and the treatment recommendations are supported with an extensive bibliography of important work in this area.
Topics: Humans; Lung Transplantation; Pneumothorax; Pulmonary Emphysema; Radiography, Thoracic
PubMed: 20955876
DOI: 10.1016/j.suc.2010.06.008 -
Scientific Reports Oct 2018Exercise limitation is common in chronic obstructive pulmonary disease (COPD). We determined the impact of pulmonary emphysema on the physiological response to exercise...
Exercise limitation is common in chronic obstructive pulmonary disease (COPD). We determined the impact of pulmonary emphysema on the physiological response to exercise independent of contemporary measures of COPD severity. Smokers 40-79 years old with COPD underwent computed tomography, pulmonary function tesing, and symptom-limited incremental exercise testing. COPD severity was quantified according to the Global Initiative for Chronic Obstructive Lung Disease (GOLD) by spirometry (GOLD 1-4); and symptom burden and exacerbation risk (GOLD A-D). Emphysema severity was quantified as the percent lung volume <-950 Hounsfield units. Regression models adjusted for age, gender, body size, smoking status, airflow limitation, symptom burden and exacerbation risk. Among 67 COPD subjects (age 67 ± 8 years; 75% male; GOLD 1-4: 11%, 43%, 30%, 16%), median percent emphysema was 11%, and peak power output (PPO) was 61 ± 32 W. Higher percent emphysema independently predicted lower PPO (-24 W per 10% increment in emphysema; 95%CI -41 to -7 W). Throughout exercise, higher percent emphysema predicted 1) higher minute ventilation, ventilatory equivalent for CO, and heart rate; and 2) lower oxy-hemoglobin saturation, and end-tidal PCO. Independent of contemporary measures of COPD severity, the extent of pulmonary emphysema predicts lower exercise capacity, ventilatory inefficiency, impaired gas-exchange and increased heart rate response to exercise.
Topics: Aged; Exercise Tolerance; Female; Heart Rate; Humans; Male; Middle Aged; Pulmonary Disease, Chronic Obstructive; Pulmonary Emphysema; Respiratory Function Tests; Smoking; Tomography, X-Ray Computed
PubMed: 30356114
DOI: 10.1038/s41598-018-34014-5 -
Anais Da Academia Brasileira de Ciencias Dec 2011Several distinct stimuli can be used to reproduce histological and functional features of human emphysema, a leading cause of disability and death. Since cigarette smoke... (Comparative Study)
Comparative Study Review
Several distinct stimuli can be used to reproduce histological and functional features of human emphysema, a leading cause of disability and death. Since cigarette smoke is the main cause of emphysema in humans, experimental researches have attempted to reproduce this situation. However, this is an expensive and cumbersome method of emphysema induction, and simpler, more efficacious alternatives have been sought. Among these approaches, elastolytic enzymes have been widely used to reproduce some characteristics of human cigarette smoke-induced disease, such as: augmentation of airspaces, inflammatory cell influx into the lungs, and systemic inflammation. Nevertheless, the use of elastase-induced emphysema models is still controversial, since the disease pathways involved in elastase induction may differ from those occurring in smoke-induced emphysema. This indicates that the choice of an emphysema model may impact the results of new therapies or drugs being tested. The aim of this review is to compare the mechanisms of disease induction in smoke and elastase emphysema models, to describe the differences among various elastase models, and to establish the advantages and disadvantages of elastase-induced emphysema models. More studies are required to shed light on the mechanisms of elastase-induced emphysema.
Topics: Animals; Disease Models, Animal; Pancreatic Elastase; Pulmonary Emphysema; Smoking
PubMed: 22159348
DOI: 10.1590/s0001-37652011005000039