From treatable traits to GETomics in airway disease: moving towards clinical practice
The treatable traits approach represents a strategy for patient management. It is based on the identification of characteristics susceptible to treatments or predictive of treatment response in each individual patient. With the objective of accelerating progress in research and clinical practice relating to such a treatable traits approach, the Portraits event was convened in Barcelona, Spain, in November 2022. Here, while reporting the key concepts that emerged from the discussions during the meeting, we review the current state of the art related to treatable traits and chronic respiratory diseases management, and we des...
Source: European Respiratory Review - January 17, 2024 Category: Respiratory Medicine Authors: Papi, A., Faner, R., Pavord, I., Baraldi, F., McDonald, V. M., Thomas, M., Miravitlles, M., Roche, N., Agusti, A. Tags: Respiratory clinical practice Reviews Source Type: research
The evolution of in vitro models of lung fibrosis: promising prospects for drug discovery
Lung fibrosis is a complex process, with unknown underlying mechanisms, involving various triggers, diseases and stimuli. Different cell types (epithelial cells, endothelial cells, fibroblasts and macrophages) interact dynamically through multiple signalling pathways, including biochemical/molecular and mechanical signals, such as stiffness, affecting cell function and differentiation. Idiopathic pulmonary fibrosis (IPF) is the most common fibrosing interstitial lung disease (fILD), characterised by a notably high mortality. Unfortunately, effective treatments for advanced fILD, and especially IPF and non-IPF progressive f...
Source: European Respiratory Review - January 17, 2024 Category: Respiratory Medicine Authors: Kolanko, E., Cargnoni, A., Papait, A., Silini, A. R., Czekaj, P., Parolini, O. Tags: Lung biology and experimental studies Reviews Source Type: research
From the microscopic to the macroscopic: clinical-radiological-pathological correlation in pulmonary hypertension
Extract
Pulmonary hypertension (PH) is defined as the presence of a mean pulmonary arterial pressure >20 mmHg [1]. This simple haemodynamic definition encompasses a heterogenous collection of conditions. It is now appreciated that although treatable forms of PH are relatively rare, PH itself is not an uncommon entity, affecting ~1% of the global population [1]. Current international guidelines describe five classification groups: group 1 (pulmonary arterial hypertension (PAH)), group 2 (PH associated with left heart disease), group 3 (PH associated with lung disease), group 4 (PH associated with pulmonary arterial ...
Source: European Respiratory Review - December 20, 2023 Category: Respiratory Medicine Authors: Condliffe, R., Dorfmüller, P., Gopalan, D., Sitbon, O., Vonk Noordegraaf, A. Tags: Editorials Source Type: research
Selection of potential targets for stratifying congenital pulmonary airway malformation patients with molecular imaging: is MUC1 the one?
This study provides an extensive overview of all known potential targets in CPAM that might identify those patients at risk for malignancy and conducted the first step towards validation, identifying MUC1 as the most promising target. (Source: European Respiratory Review)
Source: European Respiratory Review - December 20, 2023 Category: Respiratory Medicine Authors: van Horik, C., Zuidweg, M. J. P., Boerema-de Munck, A., Buscop-van Kempen, M., Brosens, E., Vahrmeijer, A. L., von der Thüsen, J. H., Wijnen, R. M. H., Rottier, R. J., Tummers, W. S. F. J., Schnater, J. M. Tags: Genetics, Paediatric pulmonology Reviews Source Type: research
Clinical-radiological-pathological correlation in chronic thromboembolic pulmonary hypertension
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and potentially life-threatening complication of acute pulmonary embolism. It is characterised by persistent fibro-thrombotic pulmonary vascular obstructions and elevated pulmonary artery pressure leading to right heart failure. The diagnosis is based on two steps, as follows: 1) suspicion based on symptoms, echocardiography and ventilation/perfusion scan and 2) confirmation with right heart catheterisation, computed tomography pulmonary angiography and, in most cases, digital subtraction angiography. The management of CTEPH requires a multimodal approach, inv...
Source: European Respiratory Review - December 20, 2023 Category: Respiratory Medicine Authors: Verbelen, T., Godinas, L., Dorfmüller, P., Gopalan, D., Condliffe, R., Delcroix, M. Tags: Lung imaging, Pulmonary vascular disease Clinical-radiological-pathological correlation in pulmonary hypertension Source Type: research
Clinical-radiological-pathological correlation in pulmonary arterial hypertension
Pulmonary hypertension (PH) is defined by the presence of a mean pulmonary arterial pressure >20 mmHg. Current guidelines describe five groups of PH with shared pathophysiological and clinical features. In this paper, the first of a series covering all five PH classification groups, the clinical, radiological and pathological features of pulmonary arterial hypertension (PAH) will be reviewed. PAH may develop in the presence of associated medical conditions or a family history, following exposure to certain medications or drugs, or may be idiopathic in nature. Although all forms of PAH share common histopathological...
Source: European Respiratory Review - December 20, 2023 Category: Respiratory Medicine Authors: Condliffe, R., Durrington, C., Hameed, A., Lewis, R. A., Venkateswaran, R., Gopalan, D., Dorfmüller, P. Tags: Lung imaging, Pulmonary vascular disease Clinical-radiological-pathological correlation in pulmonary hypertension Source Type: research
Clinical-radiological-pathological correlation in pulmonary hypertension with unclear and/or multifactorial mechanisms
Ever since the second world symposium on pulmonary hypertension (PH) held in Evian, France, in 1998, PH has been classified into five major clinical groups. Group 5 PH includes a variety of distinct conditions with unclear and/or multifactorial underlying pathologies. Management of these patients is challenging as the number of patients within these groups is often small, not all individuals with certain underlying conditions are affected by PH and patients exhibit distinct symptoms due to different underlying diseases. Studies and clinical trials in these groups are largely lacking and mostly restricted to case series and...
Source: European Respiratory Review - December 20, 2023 Category: Respiratory Medicine Authors: Lichtblau, M., Mayer, L., Gopalan, D., Dorfmüller, P., Ulrich, S. Tags: Lung imaging, Pulmonary vascular disease Clinical-radiological-pathological correlation in pulmonary hypertension Source Type: research
Balancing the value and risk of exercise-based therapy post-COVID-19: a narrative review
This article addresses these concerns and proposes that an individually prescribed, symptom-titrated exercise-based intervention may be of value to individuals following infection with severe acute respiratory syndrome coronavirus 2. (Source: European Respiratory Review)
Source: European Respiratory Review - December 20, 2023 Category: Respiratory Medicine Authors: Singh, S. J., Daynes, E., McAuley, H. J. C., Raman, B., Greening, N. J., Chalder, T., Elneima, O., Evans, R. A., Bolton, C. E. Tags: Respiratory clinical practice, Respiratory infections and tuberculosis Reviews Source Type: research
Treatable traits in asthma during pregnancy: a call for a shift towards a precision-based management approach
Asthma is the most common chronic medical condition in pregnancy. Asthma exacerbations in pregnancy are unpredictable, and are associated with adverse maternal and fetal perinatal outcomes such as preterm birth and low birthweight. Goals of asthma management in pregnancy are to establish effective asthma control and prevent exacerbations. Optimising the management of asthma in pregnancy is an important goal of practice and future research.
Treatable traits is a precision medicine paradigm proposed for the management of airways diseases, which holistically addresses the complexity and heterogeneity of airways disease. It is...
Source: European Respiratory Review - December 20, 2023 Category: Respiratory Medicine Authors: Joshi, E., Gibson, P. G., McDonald, V. M., Murphy, V. E. Tags: Asthma and allergy Reviews Source Type: research
Surgical and bronchoscopic pulmonary function-improving procedures in lung emphysema
COPD is a highly prevalent, chronic and irreversible obstructive airway disease without curative treatment. Standard therapeutic strategies, both non-pharmacological and pharmacological, have only limited effects on lung function parameters of patients with severe disease. Despite optimal pharmacological treatment, many patients with severe COPD still have a high burden of dyspnoea and a poor quality of life. If these patients have severe lung emphysema, with hyperinflation as the driver of symptoms and exercise intolerance, lung volume reduction may be an effective treatment with a significant impact on lung function, exe...
Source: European Respiratory Review - December 20, 2023 Category: Respiratory Medicine Authors: Everaerts, S., Vandervelde, C. M., Shah, P., Slebos, D.-J., Ceulemans, L. J. Tags: COPD and smoking Nonpharmacological interventions in COPD: state of the art and future directions Source Type: research
Nine controversial questions about augmentation therapy for alpha-1 antitrypsin deficiency: a viewpoint
Augmentation therapy with intravenous alpha-1 antitrypsin is the only specific treatment for alpha-1 antitrypsin deficiency (AATD)-associated emphysema. This treatment has been available and remained basically unchanged for more than 35 years, but many questions persist regarding its indications, regimen of administration and efficacy. Because AATD is a rare disease, it has not been possible to conduct randomised, placebo-controlled trials that are adequately powered for the usual outcomes analysed in non-AATD-related COPD, such as lung function decline, exacerbations, symptoms or quality of life. New outcomes such as...
Source: European Respiratory Review - December 6, 2023 Category: Respiratory Medicine Authors: Miravitlles, M., Anzueto, A., Barrecheguren, M. Tags: Respiratory clinical practice Reviews Source Type: research
Global birth prevalence of Robin sequence in live-born infants: a systematic review and meta-analysis
This study describes the global birth prevalence of RS and investigates whether prevalence estimates differ by geographical location, ethnicity or study data source (registry versus non-registry data). The protocol was prospectively registered with PROSPERO.
Databases were searched using keywords and subject terms for "Robin sequence", "epidemiology", "incidence" and "birth prevalence". Meta-analysis was performed fitting random effects models with arcsine transformation.
From 34 eligible studies (n=2722 RS cases), pooled birth prevalence was 9.5 per 100 000 live births (95% CI 7.1–12.1) with statistical heterogeneit...
Source: European Respiratory Review - December 6, 2023 Category: Respiratory Medicine Authors: Wright, M., Cortina-Borja, M., Knowles, R., Urquhart, D. S. Tags: Paediatric pulmonology Reviews Source Type: research
Airway ciliated cells in adult lung homeostasis and COPD
Cilia are organelles emanating from the cell surface, consisting of an axoneme of microtubules that extends from a basal body derived from the centrioles. They are either isolated and nonmotile (primary cilia), or grouped and motile (motile cilia). Cilia are at the centre of fundamental sensory processes and are involved in a wide range of human disorders. Pulmonary cilia include motile cilia lining the epithelial cells of the conductive airways to orchestrate mucociliary clearance, and primary cilia found on nondifferentiated epithelial and mesenchymal cells acting as sensors and cell cycle keepers. Whereas cilia are esse...
Source: European Respiratory Review - December 6, 2023 Category: Respiratory Medicine Authors: Petit, L. M. G., Belgacemi, R., Ancel, J., Saber Cherif, L., Polette, M., Perotin, J.-M., Spassky, N., Pilette, C., Al Alam, D., Deslee, G., Dormoy, V. Tags: COPD and smoking, Lung structure and function Reviews Source Type: research
Investigating the prognostic value of digital mobility outcomes in patients with chronic obstructive pulmonary disease: a systematic literature review and meta-analysis
Conclusion:
Daily step count and gait speed are negatively associated with mortality risk and other important outcomes in people with COPD and therefore may have value as prognostic indicators in clinical trials, but the quantity and quality of evidence is limited. Larger studies with consistent methodologies are called for. (Source: European Respiratory Review)
Source: European Respiratory Review - November 22, 2023 Category: Respiratory Medicine Authors: Buttery, S. C., Williams, P. J., Alghamdi, S. M., Philip, K. E. J., Perkins, A., Kallis, C., Quint, J. K., Polkey, M. I., Breuls, S., Buekers, J., Chynkiamis, N., Delgado-Ortiz, L., Demeyer, H., Frei, A., Garcia-Aymerich, J., Gimeno-Santos, E., Koch, S., Tags: COPD and smoking Reviews Source Type: research
Molecular monitoring of lung allograft health: is it ready for routine clinical use?
Maintenance of long-term lung allograft health in lung transplant recipients (LTRs) requires a fine balancing act between providing sufficient immunosuppression to reduce the risk of rejection whilst at the same time not over-immunosuppressing individuals and exposing them to the myriad of immunosuppressant drug side-effects that can cause morbidity and mortality. At present, lung transplant physicians only have limited and rather blunt tools available to assist them with this task. Although therapeutic drug monitoring provides clinically useful information about single time point and longitudinal exposure of LTRs to immun...
Source: European Respiratory Review - November 22, 2023 Category: Respiratory Medicine Authors: Pradere, P., Zajacova, A., Bos, S., Le Pavec, J., Fisher, A. Tags: Respiratory clinical practice Reviews Source Type: research
