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The European Respiratory Journal Oct 2022Severe alpha-1-antitrypsin deficiency (AATD), phenotype PiZZ, is a risk factor for pulmonary emphysema and liver disease, but its effect on cancer risk is unknown. Our...
BACKGROUND
Severe alpha-1-antitrypsin deficiency (AATD), phenotype PiZZ, is a risk factor for pulmonary emphysema and liver disease, but its effect on cancer risk is unknown. Our aim was to evaluate the risk and the risk factors for incident cancer in PiZZ individuals compared with the general population with known smoking habits.
METHODS
A longitudinal study of PiZZ individuals (n=1595) from the Swedish National AATD Register, and controls (n=5999) from Swedish population-based cohorts. Data on cancer and mortality were obtained by cross-linkage with national registers. Individuals who had undergone lung transplantation (n=10) and those with a cancer diagnosis within 5 years prior to inclusion (n=63) were excluded. The risk factors for developing cancer were analysed using proportional hazards and Fine-Gray regression models, adjusting for age, sex, smoking habits and the presence of liver disease.
RESULTS
The median follow-up time was 17 years (interquartile range 11 years) for the whole study population. The incidence rates of hepatic and non-hepatic cancer per 1000 person-years were 1.6 (95% CI 1.1-2.3) and 8.5 (95% CI 7.2-10.0), respectively, for the PiZZ individuals, and 0.1 (95% CI 0.04-0.2) and 6.6 (95% CI 6.0-7.1), respectively, for the controls. The adjusted hazard ratios for hepatic and for non-hepatic cancer were 23.4 (95% CI 9.9-55.4) and 1.3 (95% CI 1.1-1.5), respectively, in the PiZZ individuals compared with the controls.
CONCLUSION
These results suggest that individuals with severe AATD may have an increased risk of developing both hepatic and non-hepatic cancer, compared with the general population.
Topics: Humans; Longitudinal Studies; alpha 1-Antitrypsin Deficiency; Pulmonary Emphysema; Smoking; Risk Factors; Neoplasms
PubMed: 35361631
DOI: 10.1183/13993003.03200-2021 -
International Journal of Chronic... 2020Alpha-1 Antitrypsin Deficiency (AATD) is a rare genetic condition that predisposes patients to lung and liver disease and is often underdiagnosed due to incomplete... (Review)
Review
Alpha-1 Antitrypsin Deficiency (AATD) is a rare genetic condition that predisposes patients to lung and liver disease and is often underdiagnosed due to incomplete diagnosis of chronic obstructive pulmonary disease (COPD) and asthma. Improvements in physician awareness have been made, but better strategies for both diagnosis and management are still required. The only current disease-modifying therapy for AATD is the infusion of the missing Alpha-1 Antitrypsin (AAT) protein, which can slow progression of emphysema. However, AAT treatment can impact patient freedom and quality of life due to the need for weekly intravenous infusions. A symposium was held to discuss patient-centric aspects of care that have impact on the lives of patients with AATD, including exacerbations of their lung disease, self-administration of intravenous AAT therapy and pulmonary rehabilitation. Intravenous self-infusion of drugs is an established treatment strategy for patients with a variety of conditions and can improve patient quality of life, freedom and mental well-being. Experience from these areas show that patients typically manage their treatment well and without complications. When applied to AATD, training patients to self-infuse therapy can be successful, but formal guidelines would be beneficial. In addition to pharmacological intervention, individualized pulmonary rehabilitation, exercise and educational programs can encourage health-enhancing patient behavior and further improve patient quality of life. However, differences in skeletal muscle adaptations to pulmonary rehabilitation exercise regimens have been observed between patients with AATD and non-AATD COPD, highlighting the need to develop training programs specifically designed for patients with AATD.
Topics: Humans; Pulmonary Disease, Chronic Obstructive; Pulmonary Emphysema; Quality of Life; Treatment Outcome; alpha 1-Antitrypsin; alpha 1-Antitrypsin Deficiency
PubMed: 33328731
DOI: 10.2147/COPD.S276773 -
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 -
Chest Apr 2022Most pulmonary conditions reduce FVC, but studies of patients with combined pulmonary fibrosis and emphysema demonstrate that reductions in FVC are less than expected...
BACKGROUND
Most pulmonary conditions reduce FVC, but studies of patients with combined pulmonary fibrosis and emphysema demonstrate that reductions in FVC are less than expected when these two conditions coexist clinically.
RESEARCH QUESTION
Do interstitial lung abnormalities (ILAs), chest CT imaging findings that may suggest an early stage of pulmonary fibrosis in individuals with undiagnosed disease, affect the association between emphysema and FVC?
STUDY DESIGN AND METHODS
Measures of ILA and emphysema were available for 9,579 and 5,277 participants from phases 1 (2007-2011) and 2 (2012-2016) of the Genetic Epidemiology of Chronic Obstructive Pulmonary Disease Study (COPDGene), respectively. ILA were defined by Fleischner Society guidelines. Adjusted linear regression models were used to assess the associations and interactions among ILA, emphysema, measures of spirometry, and lung function.
RESULTS
ILA were present in 528 (6%) and 580 (11%) of participants in phases 1 and 2 of COPDGene, respectively. ILA modified the association between emphysema and FVC (P < .0001 for interaction) in both phases. In phase 1, in those without ILA, a 5% increase in emphysema was associated with a reduction in FVC (-110 mL; 95% CI, -121 to -100 mL; P < .0001); however, in those with ILA, it was not (-11 mL; 95% CI, -53 to 31; P = .59). In contrast, no interaction was found between ILA and emphysema on total lung capacity or on diffusing capacity of carbon monoxide.
INTERPRETATION
The presence of ILA attenuates the reduction in FVC associated with emphysema.
Topics: Emphysema; Humans; Lung; Pulmonary Emphysema; Pulmonary Fibrosis; Respiratory System Abnormalities; Smokers; Spirometry
PubMed: 34742688
DOI: 10.1016/j.chest.2021.10.034 -
BMC Pulmonary Medicine Jun 2023Pulmonary hypertension (PH) is a common complication of combined pulmonary fibrosis and emphysema (CPFE). Whether the incidence of PH is increased in CPFE compared with... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND AIM
Pulmonary hypertension (PH) is a common complication of combined pulmonary fibrosis and emphysema (CPFE). Whether the incidence of PH is increased in CPFE compared with pure pulmonary fibrosis or emphysema remains unclear. This meta-analysis aimed to evaluate the risk of PH in patients with CPFE compared to those with IPF or COPD/emphysema.
METHODS
We searched the PubMed, Embase, Cochrane Library, and CNKI databases for relevant studies focusing on the incidence of PH in patients with CPFE and IPF or emphysema. Pooled odds ratios (ORs) and standard mean differences (SMD) with 95% confidence intervals (95% CIs) were used to evaluate the differences in the clinical characteristics presence and severity of PH between patients with CPFE, IPF, or emphysema. The survival impact of PH in patients with CPFE was assessed using hazard ratios (HRs).
RESULTS
A total of 13 eligible studies were included in the meta-analysis, involving 560, 720, and 316 patients with CPFE, IPF, and emphysema, respectively. Patients with CPFE had an increased PH risk with a higher frequency of pulmonary hypertension and higher estimated systolic pulmonary artery pressure (esPAP), compared with those with IPF (OR: 2.66; 95% CI: 1.55-4.57; P < 0.01; SMD: 0.86; 95% CI: 0.52-1.19; P < 0.01) or emphysema (OR: 3.19; 95% CI: 1.42-7.14; P < 0.01; SMD: 0.73; 95% CI: 0.50-0.96; P < 0.01). In addition, the patients with CPFE combined with PH had a poor prognosis than patients with CPFE without PH (HR: 6.16; 95% CI: 2.53-15.03; P < 0.01).
CONCLUSIONS
Our meta-analysis showed that patients with CPFE were associated with a significantly higher risk of PH compared with those with IPF or emphysema alone. The presence of PH was a poor predictor of mortality.
Topics: Humans; Pulmonary Fibrosis; Hypertension, Pulmonary; Pulmonary Emphysema; Lung; Emphysema; Fibrosis; Retrospective Studies
PubMed: 37344866
DOI: 10.1186/s12890-023-02425-4 -
Zhong Nan Da Xue Xue Bao. Yi Xue Ban =... May 2022Pulmonary emphysema is one of phenotypes of lung disease that can progress to chronic obstructive pulmonary disease (COPD). The pathogenesis is unknown, which may be... (Review)
Review
Pulmonary emphysema is one of phenotypes of lung disease that can progress to chronic obstructive pulmonary disease (COPD). The pathogenesis is unknown, which may be closely related to smoking, infection, gene mutation, and air pollution. Pathological features of emphysema include the decreased airway elasticity at the distal end of the bronchioles and over-hyperinflation. As the incidence and mortality of COPD increase, emphysema has become the focus of research. The efficacy of drugs for COPD is limited, and currently the treatment of emphysema mainly depends on surgery. Since the concept of lung volume reduction surgery was proposed in the 1990s, many studies have confirmed improvement of lung function and exercise capacity, but the large wound, high mortality, and high cost have reduced its benefits. Lung volume reduction surgery with bronchoscopy technology, including valves, coils, sclerosants, vapor thermal ablation, etc., has the advantages of minimal invasive and decreased mortality under the basis of effectiveness. This article aims to review the current status of interventional therapy for pulmonary emphysema with bronchoscopy, compare and summarize the characteristics and applicable populations of interventional therapy, and provide theoretical support for patients' early referral and clinicians' decision-making.
Topics: Bronchoscopy; Emphysema; Humans; Pneumonectomy; Pulmonary Disease, Chronic Obstructive; Pulmonary Emphysema
PubMed: 35753737
DOI: 10.11817/j.issn.1672-7347.2022.210120 -
European Journal of Radiology Jul 2024We determine and compare the prevalence, subtypes, severity, and risk factors for emphysema assessed by low-dose CT(LDCT) in Chinese and Dutch general populations.
PURPOSE
We determine and compare the prevalence, subtypes, severity, and risk factors for emphysema assessed by low-dose CT(LDCT) in Chinese and Dutch general populations.
METHODS
This cross-sectional study included LDCT scans of 1143 participants between May and October 2017 from a Chinese Cohort study and 1200 participants with same age range and different smoking status between May and October 2019 from a Dutch population-based study. An experienced radiologist visually assessed the scans for emphysema presence (≥trace), subtype, and severity. Logistic regression analyses, overall and stratified by smoking status, were performed and adjusted for fume exposure, demographic and smoking data.
RESULTS
The Chinese population had a comparable proportion of women to the Dutch population (54.9 % vs 58.9 %), was older (61.7 ± 6.3 vs 59.8 ± 8.1), included more never smokers (66.4 % vs 38.3 %), had a higher emphysema prevalence ([58.8 % vs 39.7 %], adjusted odds ratio, aOR = 2.06, 95 %CI = 1.68-2.53), and more often had centrilobular emphysema (54.8 % vs 32.8 %, p < 0.001), but no differences in emphysema severity. After stratification, only in never smokers an increased odds of emphysema was observed in the Chinese compared to the Dutch (aOR = 2.55, 95 %CI = 1.95-3.35). Never smokers in both populations shared older age (aOR = 1.59, 95 %CI = 1.25-2.02 vs 1.26, 95 %CI = 0.97-1.64) and male sex (aOR = 1.50, 95 %CI = 1.02-2.22 vs 1.93, 95 %CI = 1.26-2.96) as risk factors for emphysema.
CONCLUSIONS
Only never smokers had a higher prevalence of mainly centrilobular emphysema in the Chinese general population compared to the Dutch after adjusting for confounders, indicating that factors other than smoking, age and sex contribute to presence of CT-defined emphysema.
Topics: Humans; Female; Male; Prevalence; Middle Aged; Netherlands; Pulmonary Emphysema; Cross-Sectional Studies; Tomography, X-Ray Computed; China; Risk Factors; Aged; Smoking; Severity of Illness Index; East Asian People
PubMed: 38761443
DOI: 10.1016/j.ejrad.2024.111503 -
Respiratory Research May 2017Chronic obstructive pulmonary disease (COPD) is foremost among the non-reversible fatal ailments where exposure to tobacco/biomass-smoke and aging are the major risk... (Review)
Review
Chronic obstructive pulmonary disease (COPD) is foremost among the non-reversible fatal ailments where exposure to tobacco/biomass-smoke and aging are the major risk factors for the initiation and progression of the obstructive lung disease. The role of smoke-induced inflammatory-oxidative stress, apoptosis and cellular senescence in driving the alveolar damage that mediates the emphysema progression and severe lung function decline is apparent, although the central mechanism that regulates these processes was unknown. To fill in this gap in knowledge, the central role of proteostasis and autophagy in regulating chronic lung disease causing mechanisms has been recently described. Recent studies demonstrate that cigarette/nicotine exposure induces proteostasis/autophagy-impairment that leads to perinuclear accumulation of polyubiquitinated proteins as aggresome-bodies, indicative of emphysema severity. In support of this concept, autophagy inducing FDA-approved anti-oxidant drugs control tobacco-smoke induced inflammatory-oxidative stress, apoptosis, cellular senescence and COPD-emphysema progression in variety of preclinical models. Hence, we propose that precise and early detection of aggresome-pathology can allow the timely assessment of disease severity in COPD-emphysema subjects for prognosis-based intervention. While intervention with autophagy-inducing drugs is anticipated to reduce alveolar damage and lung function decline, resulting in a decrease in the current mortality rates in COPD-emphysema subjects.
Topics: Animals; Autophagy; Evidence-Based Medicine; Humans; Lung; Male; Nicotine; Prognosis; Pulmonary Disease, Chronic Obstructive; Pulmonary Emphysema; Smoke; Smoking; Nicotiana
PubMed: 28472967
DOI: 10.1186/s12931-017-0560-7 -
Jornal Brasileiro de Pneumologia :... 2015
Topics: Female; Humans; Lung; Male; Pulmonary Disease, Chronic Obstructive; Pulmonary Emphysema
PubMed: 26785955
DOI: 10.1590/S1806-37562015000600001 -
European Radiology Experimental Nov 2020Differentiating combined pulmonary fibrosis with emphysema (CPFE) from pure emphysema can be challenging on high-resolution computed tomography (HRCT). This has...
BACKGROUND
Differentiating combined pulmonary fibrosis with emphysema (CPFE) from pure emphysema can be challenging on high-resolution computed tomography (HRCT). This has antifibrotic therapy implications.
METHODS
Twenty patients with suspected CPFE underwent late gadolinium-enhanced (LGE) thoracic magnetic resonance imaging (LGE-MRI) and HRCT. Data from twelve healthy control subjects from a previous study who underwent thoracic LGE-MRI were included for comparison. Quantitative LGE signal intensity (SI) was retrospectively compared in regions of fibrosis and emphysema in CPFE patients to similar lung regions in controls. Qualitative comparisons for the presence/extent of reticulation, honeycombing, and traction bronchiectasis between LGE-MRI and HRCT were assessed by two readers in consensus.
RESULTS
There were significant quantitative differences in fibrosis SI compared to emphysema SI in CPFE patients (25.8, IQR 18.4-31.0 versus 5.3, IQR 5.0-8.1, p < 0.001). Significant differences were found between LGE-MRI and HRCT in the extent of reticulation (12.5, IQR 5.0-20.0 versus 25.0, IQR 15.0-26.3, p = 0.038) and honeycombing (5.0, IQR 0.0-10.0 versus 20.0, IQR 10.6-20.0, p = 0.001) but not traction bronchiectasis (10.0, IQR 5-15 versus 15.0, IQR 5-15, p = 0.878). Receiver operator curve analysis of fibrosis SI compared to similarly located regions in control subjects showed an area under the curve of 0.82 (p = 0.002). A SI cutoff of 19 yielded a sensitivity of 75% and specificity of 86% in differentiating fibrosis from similarly located regions in control subjects.
CONCLUSION
LGE-MRI can differentiate CPFE from pure emphysema and may be a useful adjunct test to HRCT in patients with suspected CPFE.
Topics: Adult; Aged; Case-Control Studies; Contrast Media; Diagnosis, Differential; Female; Gadolinium; Humans; Idiopathic Pulmonary Fibrosis; Magnetic Resonance Imaging; Male; Middle Aged; Pulmonary Emphysema; Retrospective Studies; Sensitivity and Specificity; Tomography, X-Ray Computed
PubMed: 33141269
DOI: 10.1186/s41747-020-00187-w