The world of rare interstitial lung diseases
(ILDs) is diverse and complex. Diagnosis and therapy usually pose challenges. This review describes a selection of rare and ultrarare ILDs including pulmonary alveolar proteinosis, pulmonary alveolar microlithiasis and pleuroparenchymal fibroelastosis. In addition, monogenic ILDs or ILDs in congenital syndromes and various multiple cystic lung diseases will be discussed. All these conditions are part of the scope of the European Reference Network on rare respiratory diseases (ERN-LUNG). Epidemiology, pathogenesis, diagnostics and treatment of each disease are presented. (Source: European Respiratory Review)
Source: European Respiratory Review - February 7, 2023 Category: Respiratory Medicine Authors: Buschulte, K., Cottin, V., Wijsenbeek, M., Kreuter, M., Diesler, R. Tags: Interstitial and orphan lung disease The world of rare lung diseases Source Type: research
Highlights from the International Chronic Thromboembolic Pulmonary Hypertension Congress 2021
This report summarises current knowledge and presents key messages from the International CTEPH Conference, Bad Nauheim, Germany, 2021. Sessions were dedicated to 1) disease definition; 2) pathophysiology, including the impact of the hypertrophied bronchial circulation, right ventricle (dys)function, genetics and inflammation; 3) diagnosis, early after acute pulmonary embolism, using computed tomography and perfusion techniques, and supporting the selection of appropriate therapies; 4) surgical treatment, pulmonary endarterectomy for proximal and distal disease, and peri-operative management; 5) percutaneous approach or ba...
Source: European Respiratory Review - February 7, 2023 Category: Respiratory Medicine Authors: Simonneau, G., Fadel, E., Vonk Noordegraaf, A., Toshner, M., Lang, I. M., Klok, F. A., McInnis, M. C., Screaton, N., Madani, M. M., Martinez, G., Salaunkey, K., Jenkins, D. P., Matsubara, H., Brenot, P., Hoeper, M. M., Ghofrani, H. A., Jaïs, X., W Tags: Interstitial and orphan lung disease The world of rare lung diseases Source Type: research
European Respiratory Review, list of peer reviewers 2022
The European Respiratory Review is voluntarily reviewed. We are most grateful to the hard work and dedication of those listed below, who reviewed articles for the ERR in 2022. (Source: European Respiratory Review)
Source: European Respiratory Review - January 25, 2023 Category: Respiratory Medicine Tags: COPD and smoking Acknowledgement Source Type: research
The role of confocal laser endomicroscopy in pulmonary medicine
Accurate diagnosis and subsequent therapeutic options in pulmonary diseases mainly rely on imaging methods and histological assessment. However, imaging examinations are hampered by the limited spatial resolution of images and most procedures that are related to histological assessment are invasive with associated complications. As a result, a high-resolution imaging technology – confocal laser endomicroscopy (CLE), which is at the forefront and enables real-time microscopic visualisation of the morphologies and architectures of tissues or cells – has been developed to resolve the clinical dilemma pertaining to...
Source: European Respiratory Review - January 25, 2023 Category: Respiratory Medicine Authors: Tian, S., Huang, H., Zhang, Y., Shi, H., Dong, Y., Zhang, W., Bai, C. Tags: Lung imaging, Respiratory clinical practice Reviews Source Type: research
Targeting interleukin-33 and thymic stromal lymphopoietin pathways for novel pulmonary therapeutics in asthma and COPD
Interleukin-33 (IL-33) and thymic stromal lymphopoietin (TSLP) are alarmins that are released upon airway epithelial injury from insults such as viruses and cigarette smoke, and play critical roles in the activation of immune cell populations such as mast cells, eosinophils and group 2 innate lymphoid cells. Both cytokines were previously understood to primarily drive type 2 (T2) inflammation, but there is emerging evidence for a role for these alarmins to additionally mediate non-T2 inflammation, with recent clinical trial data in asthma and COPD cohorts with non-T2 inflammation providing support. Currently available trea...
Source: European Respiratory Review - January 25, 2023 Category: Respiratory Medicine Authors: Calderon, A. A., Dimond, C., Choy, D. F., Pappu, R., Grimbaldeston, M. A., Mohan, D., Chung, K. F. Tags: COPD and smoking, Pulmonary pharmacology and therapeutics, Asthma and allergy Reviews Source Type: research
European Respiratory Review, list of peer reviewers 2022
This meta-analysis explored the safety and effectiveness of mucolytics as an add-on treatment for chronic obstructive pulmonary disease (COPD) exacerbations. Based on a pre-registered protocol and following Cochrane methods, we systematically searched for relevant randomised or quasi-randomised controlled trials (RCTs). We used the Risk of Bias v2 tool for appraising the studies and performed random-effect meta-analyses when appropriate. We assessed certainty of evidence using GRADE. This meta-analysis included 24 RCTs involving 2192 patients with COPD exacerbations, entailing at least some concerns of methodological bias....
Source: European Respiratory Review - January 25, 2023 Category: Respiratory Medicine Tags: COPD and smoking Reviews Source Type: research
Physical activity promotion interventions in chronic airways disease: a systematic review and meta-analysis
Physical inactivity is common in people with chronic airways disease (pwCAD) and associated with worse clinical outcomes and impaired quality of life. We conducted a systematic review and meta-analysis to characterise and evaluate the effectiveness of interventions promoting step-based physical activity (PA) in pwCAD. We searched for studies that included a form of PA promotion and step-count outcome measure. A random-effects model was used to determine the overall effect size using post-intervention values. 38 studies (n=32 COPD; n=5 asthma; n=1 bronchiectasis; study population: n=3777) were included. Overall, implementin...
Source: European Respiratory Review - January 25, 2023 Category: Respiratory Medicine Authors: Reilly, C., Sails, J., Stavropoulos-Kalinoglou, A., Birch, R. J., McKenna, J., Clifton, I. J., Peckham, D., Birch, K. M., Price, O. J. Tags: Respiratory clinical practice Reviews Source Type: research
Endotyping COPD: hypoxia-inducible factor-2 as a molecular "switch" between the vascular and airway phenotypes?
COPD is a heterogeneous disease with multiple clinical phenotypes. COPD endotypes can be determined by different expressions of hypoxia-inducible factors (HIFs), which, in combination with individual susceptibility and environmental factors, may cause predominant airway or vascular changes in the lung. The pulmonary vascular phenotype is relatively rare among COPD patients and characterised by out-of-proportion pulmonary hypertension (PH) and low diffusing capacity of the lung for carbon monoxide, but only mild-to-moderate airway obstruction. Its histologic feature, severe remodelling of the small pulmonary arteries, can b...
Source: European Respiratory Review - January 11, 2023 Category: Respiratory Medicine Authors: Myronenko, O., Foris, V., Crnkovic, S., Olschewski, A., Rocha, S., Nicolls, M. R., Olschewski, H. Tags: COPD and smoking, Mechanisms of lung disease Reviews Source Type: research
Role of inhaled antibiotics in the era of highly effective CFTR modulators
This article reviews the role of inhaled antibiotics in the era of CFTR modulators. (Source: European Respiratory Review)
Source: European Respiratory Review - January 11, 2023 Category: Respiratory Medicine Authors: Elborn, J. S., Blasi, F., Burgel, P.-R., Peckham, D. Tags: CF and non-CF bronchiectasis Reviews Source Type: research
A scoping review on the risk of tuberculosis in specific population groups: can we expand the World Health Organization recommendations?
Since 2015, the World Health Organization (WHO) has recommended prioritising testing and treatment of tuberculosis (TB) infection (TBI) in 11 high-risk groups. With new options emerging for TB preventive treatment, we conducted a scoping review, in consultation with the WHO's Global Tuberculosis Programme, to explore the evidence for other population groups at potentially high risk of progression to active TB. We searched six databases for preprints and articles published between 2000 and August 2022. 18 out of 33 668 screened records were included (six meta-analyses and 12 original research studies). Most were observation...
Source: European Respiratory Review - January 11, 2023 Category: Respiratory Medicine Authors: Bigio, J., Viscardi, A., Gore, G., Matteelli, A., Sulis, G. Tags: Respiratory infections and tuberculosis Reviews Source Type: research
Immune mechanisms in fibrotic pulmonary sarcoidosis
Sarcoidosis is an immune-mediated disorder. Its immunopathology has been steadily mapped out over the past few decades. Despite this, the underpinning mechanisms for progressive fibrotic sarcoidosis is an almost uncharted area. Consequently, there has been little change in the clinical management of fibrotic sarcoidosis over the decades and an unfocused search for new therapeutics. In this review, we provide a comprehensive examination of the relevant immune findings in fibrotic and/or progressive pulmonary sarcoidosis and propose a unifying mechanism for the pathobiology of fibrosis in sarcoidosis. (Source: European Respiratory Review)
Source: European Respiratory Review - December 21, 2022 Category: Respiratory Medicine Authors: Weeratunga, P., Moller, D. R., Ho, L.-P. Tags: Interstitial and orphan lung disease Reviews Source Type: research
Role of autophagy in lung diseases and ageing
The lungs face ongoing chemical, mechanical, biological, immunological and xenobiotic stresses over a lifetime. Advancing age progressively impairs lung function. Autophagy is a "housekeeping" survival strategy involved in numerous physiological and pathological processes in all eukaryotic cells. Autophagic activity decreases with age in several species, whereas its basic activity extends throughout the lifespan of most animals. Dysregulation of autophagy has been proven to be closely related to the pathogenesis of several ageing-related pulmonary diseases. This review summarises the role of autophagy in the pathogenesis o...
Source: European Respiratory Review - December 21, 2022 Category: Respiratory Medicine Authors: Zhang, Y., Zhang, J., Fu, Z. Tags: Mechanisms of lung disease Reviews Source Type: research
New antibiotics for Gram-negative pneumonia
Pneumonia is frequently encountered in clinical practice, and Gram-negative bacilli constitute a significant proportion of its aetiology, especially when it is acquired in a hospital setting. With the alarming global rise in multidrug resistance in Gram-negative bacilli, antibiotic therapy for treating patients with pneumonia is challenging and must be guided by in vitro susceptibility results. In this review, we provide an overview of antibiotics newly approved for the treatment of pneumonia caused by Gram-negative bacilli. Ceftazidime-avibactam, imipenem-relebactam and meropenem-vaborbactam have potent activity against s...
Source: European Respiratory Review - December 21, 2022 Category: Respiratory Medicine Authors: Bassetti, M., Magne, F., Giacobbe, D. R., Bini, L., Vena, A. Tags: Respiratory infections and tuberculosis Source Type: research
Patient-reported outcomes to assess dyspnoea in interstitial lung disease and pulmonary hypertension: a systematic literature review of measurement properties
Conclusion:
Four dyspnoea-specific PROMs, D12, ERS-IPF-B, KBILD-B and UCSD-SOBQ, can be recommended for use in patients with ILD, including CTD-associated ILD. Of these four, the D12, despite the limited evidence and the lack of evidence on several important domains, is also suitable for use in patients with PH, including CTD-associated PH. (Source: European Respiratory Review)
Source: European Respiratory Review - December 21, 2022 Category: Respiratory Medicine Authors: Lemmers, J. M. J., Vonk, M. C., van den Ende, C. H. M. Tags: Interstitial and orphan lung disease, Respiratory clinical practice, Pulmonary vascular disease Reviews Source Type: research
Sensitivity and complications of thoracentesis and thoracoscopy: a meta-analysis
Background
Thoracentesis and thoracoscopy are used to diagnose malignant pleural effusions (MPE). Data on how sensitivity varies with tumour type is limited.
Methods
Systematic review using PubMed was performed through August 2020 to determine the sensitivity of thoracentesis and thoracoscopy for MPE secondary to malignancy, by cancer type, and complication rates. Tests to identify sources of heterogeneity were performed. Study quality was assessed using Quality Assessment of Diagnostic Accuracy Studies (QUADAS)-2 and National Institutes of Health quality assessment tools. Publication bias was tested using funnel plots.
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Source: European Respiratory Review - December 21, 2022 Category: Respiratory Medicine Authors: Martinez-Zayas, G., Molina, S., Ost, D. E. Tags: Respiratory clinical practice, Lung cancer Reviews Source Type: research