Platelets: a potential role in chronic respiratory diseases?
Platelets are small anucleate cells known for their role in haemostasis and thrombosis. In recent years, an increasing number of observations have suggested that platelets are also immune cells and key modulators of immunity. They express different receptors and molecules that allow them to respond to pathogens, and to interact with other immune cells. Platelets were linked to the pathogenesis of some inflammatory disorders including respiratory diseases such as asthma and idiopathic pulmonary fibrosis. Here, we discuss the involvement of platelets in different immune responses, and we focus on their potential role in vari...
Source: European Respiratory Review - September 15, 2021 Category: Respiratory Medicine Authors: Chebbo, M., Duez, C., Alessi, M. C., Chanez, P., Gras, D. Tags: Lung biology and experimental studies, Mechanisms of lung disease Review Source Type: research

Impairment of hypoxic pulmonary vasoconstriction in acute respiratory distress syndrome
Acute respiratory distress syndrome (ARDS) is a serious complication of severe systemic or local pulmonary inflammation, such as caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. ARDS is characterised by diffuse alveolar damage that leads to protein-rich pulmonary oedema, local alveolar hypoventilation and atelectasis. Inadequate perfusion of these areas is the main cause of hypoxaemia in ARDS. High perfusion in relation to ventilation (V/Q<1) and shunting (V/Q=0) is not only caused by impaired hypoxic pulmonary vasoconstriction but also redistribution of perfusion from obstructed lung ve...
Source: European Respiratory Review - September 15, 2021 Category: Respiratory Medicine Authors: Gierhardt, M., Pak, O., Walmrath, D., Seeger, W., Grimminger, F., Ghofrani, H. A., Weissmann, N., Hecker, M., Sommer, N. Tags: Acute lung injury and critical care Review Source Type: research

The cystic fibrosis lung microenvironment alters antibiotic activity: causes and effects
We describe how the CF lung microenvironment, shaped by host factors (such as iron, mucus, immune mediators and oxygen availability) and the microbiota, influences antibiotic activity and varies widely between patients. A better understanding of the CF microenvironment and population diversity may thus help improve in vitro antibiotic susceptibility testing and clinical decision making, in turn increasing the success rate of antibiotic treatment. (Source: European Respiratory Review)
Source: European Respiratory Review - September 15, 2021 Category: Respiratory Medicine Authors: Van den Bossche, S., De Broe, E., Coenye, T., Van Braeckel, E., Crabbe, A. Tags: CF and non-CF bronchiectasis Review Source Type: research

Exertional ventilation/carbon dioxide output relationship in COPD: from physiological mechanisms to clinical applications
There is well established evidence that the minute ventilation (V'E)/carbon dioxide output (V'CO2) relationship is relevant to a number of patient-related outcomes in COPD. In most circumstances, an increased V'E/V'CO2 reflects an enlarged physiological dead space ("wasted" ventilation), although alveolar hyperventilation (largely due to increased chemosensitivity) may play an adjunct role, particularly in patients with coexistent cardiovascular disease. The V'E/V'CO2 nadir, in particular, has been found to be an important predictor of dyspnoea and poor exercise tolerance, even in patients with largely preserved forced exp...
Source: European Respiratory Review - September 15, 2021 Category: Respiratory Medicine Authors: Neder, J. A., Berton, D. C., Phillips, D. B., O'Donnell, D. E. Tags: COPD and smoking, Mechanisms of lung disease Ventilatory efficiency and its clinical prognostic value in cardiorespiratory disorders Source Type: research

Minute ventilation/carbon dioxide production in congenital heart disease
This review summarises various applications of how ventilatory equivalent (ventilatory efficiency or better still ventilatory inefficiency) and the minute ventilation (V'E)/carbon dioxide production (V'CO2) slope obtained from cardiopulmonary exercise testing (CPET) can be used in the diagnostic or prognostic workup of patients with congenital heart disease. The field of congenital heart disease comprises not only a very heterogeneous patient group with various heart diseases, but also various conditions in different stages of repair, as well as the different residuals seen in long-term follow-up. As such, various physiolo...
Source: European Respiratory Review - September 15, 2021 Category: Respiratory Medicine Authors: Hager, A. Tags: Mechanisms of lung disease Ventilatory efficiency and its clinical prognostic value in cardiorespiratory disorders Source Type: research

Recycling plastic: diagnosis and management of plastic bronchitis among adults
Plastic bronchitis is a rare, underdiagnosed and potentially fatal condition. It is characterised by the formation and expectoration of branching gelatinous plugs that assume the shape of the airways. These airway plugs differ from the allergic mucin that characterises allergic bronchopulmonary aspergillosis and mucoid impaction of the bronchi. Plastic bronchitis is most often encountered in the paediatric population following corrective cardiac surgery, such as the Fontan procedure. It also occurs in adults. Plastic bronchitis in adults is rare, heterogeneous in its aetiology, and can lead to respiratory distress or even ...
Source: European Respiratory Review - August 17, 2021 Category: Respiratory Medicine Authors: Ntiamoah, P., Mukhopadhyay, S., Ghosh, S., Mehta, A. C. Tags: Interstitial and orphan lung disease Review Source Type: research

Cryptogenic organising pneumonia: current understanding of an enigmatic lung disease
Organising pneumonia (OP) is currently recognised as a nonspecific lung injury response that is associated with a variety of imaging patterns obtained with high-resolution computed tomography (HRCT) of the chest and is characterised histopathologically by the presence of inflammatory cells and a connective tissue matrix within distal airspaces of the lungs. OP is associated with many conditions that include connective tissue disorders, various infections, drug reactions, hypersensitivity pneumonitis and aspiration. When OP cannot be linked to an associated condition and appears to be idiopathic, it is termed cryptogenic or...
Source: European Respiratory Review - August 17, 2021 Category: Respiratory Medicine Authors: Raghu, G., Meyer, K. C. Tags: Interstitial and orphan lung disease Review Source Type: research

Pulmonary hypertension phenotypes in patients with systemic sclerosis
Pulmonary hypertension (PH) commonly affects patients with systemic sclerosis (SSc) and is associated with significant morbidity and increased mortality. PH is a heterogenous condition and several different forms can be associated with SSc, including pulmonary arterial hypertension (PAH) resulting from a pulmonary arterial vasculopathy, PH due to left heart disease and PH due to interstitial lung disease. The incidence of pulmonary veno-occlusive disease is also increased. Accurate and early diagnosis to allow optimal treatment is, therefore, essential. Recent changes to diagnostic haemodynamic criteria at the 6th World Sy...
Source: European Respiratory Review - August 17, 2021 Category: Respiratory Medicine Authors: Haque, A., Kiely, D. G., Kovacs, G., Thompson, A. A. R., Condliffe, R. Tags: Pulmonary vascular disease Review Source Type: research

A scoping review of palliative care outcome measures in interstitial lung disease
Interstitial lung disease (ILD) confers a high mortality and symptom burden, substantially impacting quality of life. Studies evaluating palliative care in ILD are rapidly expanding. Uniform outcome measures are crucial to assessing the impact of palliative care in ILD. This scoping review evaluates existing outcome measures in general health-related quality of life (HRQoL), physical health, mental health, social health and advance care planning (ACP) domains in patients with ILD. Articles in English with quantitative assessment of at least one measure of general HRQoL, physical health, mental health, social health or ACP ...
Source: European Respiratory Review - August 3, 2021 Category: Respiratory Medicine Authors: Gersten, R. A., Moale, A. C., Seth, B., Vick, J. B., Brown, H., Eakin, M. N., Mathai, S. C., Danoff, S. K. Tags: Interstitial and orphan lung disease Review Source Type: research

Airway granulation response to lung-implantable medical devices: a concise overview
Increasing numbers of endoscopically implantable devices are implanted in the airways, such as airway stents, one-way valves and coils, to treat both malignant and benign diseases. They significantly improve patient outcomes, but their long-term effectiveness and sustainability is hampered by the reaction of the formation of granulation tissue. Factors including procedural-related tissue injury; micro-organism presence; device-related factors, such as the material, design and sizing in relation to the airway; and patient-related factors, including genetic susceptibility, comorbidities and medication use, might all effect t...
Source: European Respiratory Review - August 3, 2021 Category: Respiratory Medicine Authors: Roodenburg, S. A., Pouwels, S. D., Slebos, D.-J. Tags: Pulmonary pharmacology and therapeutics Review Source Type: research

Human pluripotent stem cells for the modelling and treatment of respiratory diseases
Respiratory diseases are among the leading causes of morbidity and mortality worldwide, representing a major unmet medical need. New chemical entities rarely make it into the clinic to treat respiratory diseases, which is partially due to a lack of adequate predictive disease models and the limited availability of human lung tissues to model respiratory disease. Human pluripotent stem cells (hPSCs) may help fill this gap by serving as a scalable human in vitro model. In addition, human in vitro models of rare genetic mutations can be generated using hPSCs. hPSC-derived epithelial cells and organoids have already shown grea...
Source: European Respiratory Review - August 3, 2021 Category: Respiratory Medicine Authors: Goldsteen, P. A., Yoseif, C., Dolga, A. M., Gosens, R. Tags: Lung biology and experimental studies Review Source Type: research

Lung transplantation for interstitial lung disease
Lung transplantation (LTx) can be a life-extending treatment option for patients with advanced and/or progressive fibrotic interstitial lung disease (ILD), especially idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis, sarcoidosis and connective tissue disease-associated ILD. IPF is now the most common indication for LTx worldwide. Several unique features in patients with ILD can impact optimal timing of referral or listing for LTx, pre- or post-transplant risks, candidacy and post-transplant management. As the epidemiology of LTx and community practices have evolved, recent literature describes out...
Source: European Respiratory Review - August 3, 2021 Category: Respiratory Medicine Authors: Kapnadak, S. G., Raghu, G. Tags: Interstitial and orphan lung disease Review Source Type: research

Tuberous sclerosis complex for the pulmonologist
Tuberous sclerosis complex (TSC) is a rare multisystem genetic disorder affecting almost all organs with no sex predominance. TSC has an autosomal-dominant inheritance and is caused by a heterozygous mutation in either the TSC1 or TSC2 gene leading to hyperactivation of the mammalian target of rapamycin (mTOR). TSC is associated with several pulmonary manifestations including lymphangioleiomyomatosis (LAM), multifocal micronodular pneumocyte hyperplasia (MMPH) and chylous effusions. LAM is a multisystem disorder characterised by cystic destruction of lung parenchyma, and may occur in either the setting of TSC (TSC-LAM) or ...
Source: European Respiratory Review - August 3, 2021 Category: Respiratory Medicine Authors: Rebaine, Y., Nasser, M., Girerd, B., Leroux, C., Cottin, V. Tags: Genetics Rare genetic interstitial lung diseases Source Type: research

Liposomal drug delivery to manage nontuberculous mycobacterial pulmonary disease and other chronic lung infections
Nontuberculous mycobacterial (NTM) pulmonary disease is a chronic respiratory infection associated with declining lung function, radiological deterioration and significantly increased morbidity and mortality. Patients often have underlying lung conditions, particularly bronchiectasis and COPD. NTM pulmonary disease is difficult to treat because mycobacteria can evade host defences and antimicrobial therapy through extracellular persistence in biofilms and sequestration into macrophages. Management of NTM pulmonary disease remains challenging and outcomes are often poor, partly due to limited penetration of antibiotics into...
Source: European Respiratory Review - July 20, 2021 Category: Respiratory Medicine Authors: Chalmers, J. D., van Ingen, J., van der Laan, R., Herrmann, J.-L. Tags: Respiratory infections and tuberculosis Review Source Type: research

Update on molecular pathology and role of liquid biopsy in nonsmall cell lung cancer
Personalised medicine, an essential component of modern thoracic oncology, has been evolving continuously ever since the discovery of the epidermal growth factor receptor and its tyrosine kinase inhibitors. Today, screening for driver alterations in patients with advanced lung adenocarcinoma as well as those with squamous cell carcinoma and no/little history of smoking is mandatory. Multiplex molecular platforms are preferred to sequential molecular testing since they are less time- and tissue-consuming. In this review, we present the latest updates on the nine most common actionable driver alterations in nonsmall cell lun...
Source: European Respiratory Review - July 20, 2021 Category: Respiratory Medicine Authors: Abdayem, P., Planchard, D. Tags: Lung cancer Thoracic oncology Source Type: research