The diagnosis of interstitial lung diseases (ILDs) can be straightforward for some experienced experts without biopsy data, but not so straightforward to others. In the context of suspected idiopathic pulmonary fibrosis (IPF), accurate non-invasive diagnosis requires recognition of the hallmark usual interstitial pneumonia (UIP) pattern on imaging, obviating lung biopsy [1, 2]. When the imaging pattern

Pulmonary arterial hypertension (PAH) is characterised by progressive obliteration of the pulmonary vasculature, culminating in right-sided heart failure (HF). In recent years, studies have observed an association between systemic metabolic dysfunction, PAH and right ventricular failure [1]. Patients with PAH have an increased prevalence of obesity [2] and type 2 diabetes [3, 4]. Interestingly, abnormal

Current diagnostic parameters estimating obstructive sleep apnoea (OSA) severity have a poor connection to the psychomotor vigilance of OSA patients. Thus, we aimed to investigate how the severity of apnoeas, hypopnoeas and intermittent hypoxaemia is associated with impaired vigilance. We retrospectively examined type I polysomnography data and corresponding psychomotor vigilance tasks (PVTs) of 743

Introduction The ex vivo lung perfusion (EVLP) technique has been developed to assess the function of marginal donor lungs and has significantly increased donor lung utilisation. EVLP has also been explored as a platform for donor lung repair through injury-specific treatments such as antibiotics or fibrinolytics. We hypothesised that actively expressed pathways shared between transplantation and EVLP

Recent studies have suggested that in patients with an idiopathic interstitial pneumonia (IIP), a probable usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT) is sufficient to diagnose idiopathic pulmonary fibrosis (IPF) without histopathology. We retrospectively compared the prognosis and time to first acute exacerbation (AE) in IIP patients with a UIP and a probable UIP pattern

Individuals with idiopathic pulmonary arterial hypertension (PAH) display reduced oral glucose tolerance. This may involve defects in pancreatic function or insulin sensitivity but this hypothesis has not been tested; moreover, fasting nutrient metabolism remains poorly described in PAH. Thus, we aimed to characterise fasting nutrient metabolism and investigated the metabolic response to hyperglycaemia

In 2018, 806 tuberculosis (TB) patients were notified in the Netherlands, which was the second lowest number recorded since notification became mandatory in 1980; only 2017 had fewer notified cases (n=783) [1]. In this century, TB notification rates in the Netherlands declined by 50%, from 9.3 patients per 100 000 population in 2001 (1492 patients) to 4.7 per 100 000 in 2018. In 2018, 77% of the TB

Pulmonary fibrosis is considered to result from recurrent alveolar epithelial injury coupled with dysfunctional alveolar wound healing mechanisms, some of which have a genetic background. Pulmonary fibrosis in the adult has not been previously associated with prolidase deficiency, an innate deficiency of amino acid metabolism. Prolidase deficiency is a new genetic cause of combined pulmonary fibrosis

We read with great interest the article by Wijnant et al . [1] in a recent issue of European Respiratory Journal investigating the longitudinal outcomes of patients with preserved ratio impaired spirometry (PRISm) in the Rotterdam cohort. PRISm is a subject of increased investigation as it is associated with respiratory symptoms, cardiovascular disease, metabolic syndrome and, quite clearly in the

In a recent paper published in the European Respiratory Journal , we studied the characteristics, trajectory and mortality of subjects with Preserved Ratio Impaired Spirometry (PRISm) in the Rotterdam Study, a prospective population-based cohort study in the Netherlands, a high-income country (HIC) [1]. In an elegant letter to the editor, P. Jackson and T. Siddharthan correctly argue that clinical

Lung disease is a frequent clinical manifestation in people living with primary immunodeficiency diseases, the most prevalent of which are common variable immunodeficiency disorders (CVID). CVID is primarily characterised by antibody deficiency, but recent definitions and diagnostic criteria recognise a much more complex pattern of immunological defects [1]. CVID can be classified into two major clinical

Though the notion of a “lung microbiome”, i.e . a complex community of microbes inhabiting and influencing the lower respiratory tract, is barely a decade old [1], there is nothing new about the idea that the lungs represent a crucial interface between us and the microbial universe we inhabit. Louis Pasteur demonstrated the presence of viable microbes in air [2], and one of his contemporaries calculated

The natural history of cystic fibrosis (CF) lung disease has been described as a complex process of obstruction, infection, and inflammation, all contributing to airways injury and eventual structural disease ( i.e. bronchiectasis). Consequences of the CF genetic abnormality begin in utero and are the basis for newborn screening for elevated levels of circulating immunoreactive trypsinogen (IRT) [1]

Air pollution has been with humankind throughout evolution ( e.g. volcanoes, forest fires), but it rapidly accelerated with the industrial revolution. Although the history of London's suffocating fogs began in the 18th century with coal power, it was the acute London fog episode of December 1952, and similar crises, that galvanised public health officials to begin to address the problem. The tremendous

There is growing recognition for the involvement of blood vessels in pulmonary diseases beyond the classical scope of pulmonary vascular medicine. As an example, reports have described a vascular phenotype of COPD, or have proposed a vanishing pulmonary capillary syndrome to underly emphysema [1, 2]. Although the number of clinical and epidemiological studies supporting such a vascular hypothesis of

Often, the past can help us more than we realise. The European Respiratory Journal has, since its early childhood about 30 years ago, demonstrated that publication of reference values for various lung function tests is something that receives priority. Although one may argue that publication of reference values for lung function tests will not yield a Nobel Prize, everyone will agree that valid and

Increasing bacterial burden in the lower airways of patients with idiopathic pulmonary fibrosis confers an increased risk of disease progression and mortality. However, it remains unclear whether this increased bacterial burden directly influences progression of fibrosis or simply reflects the magnitude of the underlying disease extent or severity. We prospectively recruited 193 patients who underwent

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often deadly complication of IPF. No focussed international guidelines for the management of AE-IPF exist. The aim of this international survey was to assess the global variability in prevention, diagnostic and treatment strategies for AE-IPF. Pulmonologists with ILD expertise were invited to participate in a survey designed by an international

Little is known about early predictors of later cystic fibrosis (CF) structural lung disease. This study examined early predictors of progressive structural lung abnormalities in children who completed the Australasian CF Bronchoalveolar Lavage (ACFBAL) clinical trial at age 5-years and participated in an observational follow-up study (CF-FAB). Eight Australian and New Zealand CF centres participated

Outdoor air pollution exposure increases chronic obstructive pulmonary disease (COPD) hospitalisations, and may contribute to COPD development. The mechanisms of harm, and the extent to which at-risk populations are more susceptible are not fully understood. Neutrophils are recruited to the lung following diesel exhaust exposure, a model of traffic-related air pollution (TRAP), but their functional

Beyond the major gene BMPR2 , several new genes predisposing to PAH have been identified during the last decade. Recently, preliminary evidence of the involvement of the KDR gene was found in a large genetic association study. We prospectively analysed the KDR gene by targeted panel sequencing in a series of 311 PAH patients referred to a clinical molecular laboratory for genetic diagnosis of PAH.

Background The multiple breath nitrogen washout (N2MBW) technique is increasingly used to assess the degree of ventilation inhomogeneity in school-aged children with lung disease. However, reference values for healthy children are currently not available. The aim of this study was to generate reference values for N2MBW outcomes in a cohort of healthy Caucasian school-aged children. Methods N2MBW data

While immunoglobulin (Ig) E is a prominent biomarker for early-onset, its levels are often elevated in non-allergic late-onset asthma. However, the pattern of IgE expression in the latter is mostly polyclonal, with specific IgEs low or below detection level albeit with an increased total IgE. In late-onset severe asthma patients, specific IgE to Staphylococcal enterotoxins (se-IgE) can frequently be

NORDSTAR is a longitudinal dataset for the study of asthma comprising 3.3 million individuals and over 50 million person-years in 4 Nordic countries. The data include diagnoses, medication dispensing, use of resources and costs, socio-demographics, and mortality.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing lung disease that leads to unrelenting dyspnoea and chronic cough, and ultimately respiratory failure [1]. IPF is characterised by a variable disease course that remains difficult to predict for an individual at diagnosis [2]. In the current era, with the advent of anti-fibrotic therapy which can slow disease progression, it is

Over the past 12 years, many genome-wide association studies (GWAS) have been conducted to identify susceptibility variants for asthma [1–5]. The Trans-National Asthma Genetic Consortium (TAGC) recently conducted a large meta-analysis of GWAS [3], identifying 878 single nucleotide polymorphisms (SNPs) associated with asthma at 18 loci. The mechanisms underlying such associations are largely unknown

The recently updated European Society of Cardiology/European Respiratory Society guidelines for acute pulmonary embolism (PE) underline the importance of appropriate long-term management of PE sequelae in the era of extended anticoagulation [1]. Chronic thromboembolic disease (CTED) is one of several conditions contributing to breathlessness in this setting, i.e. persistent pulmonary vascular obstruction

The comment by C. McCabe and colleagues provides thoughtful insights into the late sequalae of pulmonary embolism. The authors' views and suggestions, based on their clinical experience and expertise, may prove helpful in the ongoing process of reliably (and meaningfully) distinguishing chronic thromboembolic pulmonary hypertension (CTEPH) from chronic thromboembolic disease (CTED) without pulmonary

Sarcoidosis is a rare “old disease” first described over a century ago, known as a multifaceted systemic multiorgan inflammatory granulomatosis characterised by the presence of non-necrotising epithelioid granulomas [1, 2]. In many ways sarcoidosis remains a mysterious disease with numerous unsolved knowledge gaps. The still unknown aetiology/aetiologies of sarcoidosis is one of these gaps, i.e. the

Sarcoidosis is a disease characterised by granuloma formation. There is an unmet need for new treatment strategies beyond corticosteroids. The NLRP3 inflammasome pathway is expressed in innate immune cells and senses danger signals to elicit inflammatory interleukin (IL)-1β; it has recently become a druggable target. This prompted us to test the role of the NLRP3 inflammasome and IL-1β pathway in granuloma

Introduction Indoor air pollution and maternal smoking during pregnancy are associated with respiratory symptoms in infants, but little is known about the direct association with lung function or interactions with genetic risk factors. We examined associations of exposure to indoor particulate matter with a 50% cut-off aerodynamic diameter of 10 µm (PM10) and maternal smoking with infant lung function

Background The World Health Organization (WHO) recommends tuberculosis (TB) preventive treatment for high-risk groups. Isoniazid preventive therapy (IPT) has been used globally for this purpose for many years, including in pregnancy. This review assessed current knowledge about the safety of IPT in pregnancy. Methods We searched PubMed, Embase, CENTRAL, Global Health Library and HIV and TB-related

Asthma is a chronic inflammatory disease of the airways, affecting over 350 million people worldwide and placing a significant burden on healthcare providers and wider society. Approximately 5–10% of asthma patients are diagnosed with severe asthma and typically are associated with increased risk of hospitalisation from exacerbations, increased morbidity, mortality and higher asthma-associated healthcare

The underlying pathophysiological determinants of asthma and COPD are related in complex ways. Importantly however, post-bronchodilator forced expiratory volume in 1 s (FEV1) reversibility may occur in approximately 50% of COPD patients [1], whilst epidemiological [2] and magnetic resonance imaging (MRI) studies [3] suggest that, in asthma patients, FEV1 reversibility may diminish over time. As compared

We read with great interest the paper on relative and absolute lung function change in a general population aged 62–102 years in the region of Skåne, Sweden [1], since lung function research in the growing age group of older adults, especially those ≥80 years old, is indeed limited [2, 3]. Spirometry quality and interpretation in very old adults needs to be further researched, especially the use of

We thank E. Hegendörfer and J-M. Degryse for their interest and request for clarification regarding the spirometry inclusion criteria of our study as well your suggestions on avenues for future research. As stated in our paper, up to a maximum of eight consecutive breathing manoeuvres were performed with the goal of completing at least three acceptable spirograms [1]. However, there was a minority

Figures 1 and 2 in this article were published with the incorrect frailty index definitions for “frail” and “non-frail” subjects (in the key to the figures). Frail subjects were incorrectly defined as those with frailty index ≤0.21, and non-frail subjects with frailty index >0.21. The correct definitions of frail and non-frail subjects are those with frailty index >0.21 and ≤0.21, respectively, as

Tuberculosis (TB) ranks as the leading cause of death among infectious diseases in human history, claiming over a billion lives in the past two centuries alone [1, 2]. Although a number of important advances have been made to control TB in the past decade, an estimated 10 million people fell ill with TB and 1.5 million died from the disease in 2018 alone [1]. The only licensed TB vaccine, bacille Calmette–Guérin

2020 is the year of the rat. The rat is the first of the 12 Chinese zodiac signs, and represents spirit, alertness, flexibility and vitality. In respiratory medicine, we may think of rats as vectors for diseases, such as pulmonary forms of hantavirus disease or leptospirosis, and pneumonic plague. Rodent control is thus part of hygiene guidelines and the International Health Regulations. And yet, the

Positive airway pressure (PAP) is the most effective therapy for obstructive sleep apnoea (OSA), a condition affecting an estimated 936 million adults worldwide [1]. Adequate PAP adherence has been defined as 4 h of average use in most research studies. Many US insurance carriers and national payers, including the Centers for Medicaid and Medicare Services (CMS), determine coverage of PAP and supplies

Pulmonary arterial hypertension (PAH) is a rare but deadly cardiopulmonary disease, which has an unclear and multifactorial pathobiology [1]. Unfortunately, despite availability of 13 Food and Drug Administration-approved therapeutic interventions, the 5-year survival rate is just 21–64% [2]. An important clinical need exists to better understand the molecular mechanisms that lead to PAH development

With three new and three recently repurposed drugs, treatment of multidrug-resistant tuberculosis (MDR-TB) is more hopeful today than at any time since it emerged in the 1980s and 1990s [1–3]. This optimism contrasts starkly with dismal global treatment success rates of 48–56% as reported by the World Health Organization (WHO) in its annual Global Tuberculosis Reports from 2014 to 2019 [4, 5]. In low-

Introduction Continuous positive airway pressure (CPAP) is currently the treatment of choice for sleepiness in patients with obstructive sleep apnoea (OSA); however, adherence is often thought to be suboptimal. We investigated the effects of suboptimal CPAP usage on objective and subjective sleepiness parameters in patients with OSA. Material and methods In this 2-week, parallel, double-blind, randomised

Background and objectives Gefapixant has previously demonstrated efficacy in the treatment of refractory chronic cough at a high daily dose. The current investigations explore efficacy and tolerability of gefapixant, a P2X3 receptor antagonist, for the treatment of chronic cough using a dose-escalation approach. Materials and methods Two randomised, double-blind, placebo-controlled, crossover, dose-escalation

Endothelial dysfunction is a hallmark of pulmonary arterial hypertension (PAH) but there are no established methods to study pulmonary artery endothelial cells (PAECs) from living patients. We sought to culture PAECs from pulmonary artery catheter (PAC) balloons used during right-heart catheterisation (RHC) to characterise successful culture attempts and to describe PAEC behaviour. PAECs were grown

Swedish National tuberculosis (TB) guidelines recommend screening of active and latent TB (LTBI) among pregnant women (PW) from high-endemic countries or with previous exposure to possibly improve early detection and treatment. We evaluated cascade of care of a newly introduced TB screening programme of pregnant women in Stockholm county in 2016–2017. The algorithm included clinical data and Quantiferon

Rationale Studies investigating the risk of active tuberculosis (TB) in association with pregnancy have not been conclusive. We aimed to investigate this risk in a large retrospective register-based cohort study in Sweden. Methods Data from women of 15–49 years of age who had given birth in Sweden between 2005 and 2013 were extracted from the national childbirth register and linked to the national

Clinical trials suggest less hepatotoxicity and better adherence with 4 months rifampin (4R) versus 9 months isoniazid (9H) for treating latent tuberculosis infection (LTBI). Our objectives were to compare frequencies of severe hepatic adverse events and treatment completion, and direct health system costs of LTBI regimens 4R and 9H, in the general population of the province of Quebec, Canada, using

We sought to compare the effectiveness of two World Health Organization (WHO)-recommended regimens for the treatment of rifampin- or multidrug-resistant (RR/MDR) tuberculosis (TB): a standardised regimen of 9–12 months (the “shorter regimen”) and individualised regimens of ≥20 months (“longer regimens”). We collected individual patient data from observational studies identified through systematic reviews

Diabetes is associated with failure of tuberculosis (TB) treatment, but it is unclear whether this is related to genotypic drug resistance of the infecting mycobacteria. We used whole genome sequencing (WGS) to examine 1365 known drug resistance mutations in 896 Mycobacterium tuberculosis isolates from TB patients that were screened for diabetes using HbA1c testing as part of the TANDEM project [1]

Control of Mycobacterium tuberculosis transmission in high-income healthcare settings and in low tuberculosis (TB) prevalence countries remains a public health priority given the constant changes in M. tuberculosis epidemiology worldwide. Though Europe is a low prevalence area [1], TB burden among precarious and migrant populations contributes to this evolving landscape, as addressed by the action

While the Xpert MTB/RIF assay (Cepheid Sunnyvale, CA, USA) has greater than 95% sensitivity for identifying acid-fast bacilli (AFB) smear-positive tuberculosis (TB) cases, for smear-negative pulmonary tuberculosis (PTB) the sensitivity was as low as 60% [1, 2]. To address the low sensitivity of current TB diagnostics for AFB smear-negative cases, we developed a simple and label-free pathogen enrichment

We read with interest the recent study by Suissa et al. [1] published in the European Respiratory Journal . The study used population-based administrative data from the province of Quebec, Canada, to evaluate the association between inhaled corticosteroid (ICS) exposure and lung cancer risk in patients with COPD. The results of the study indicated that ICS use was not associated with lung cancer risk

We thank A.J.N. Raymakers and co-workers for their letter, which clarifies the method of data analysis used in their study of the association between inhaled corticosteroids (ICS) and lung cancer incidence in COPD [1]. Indeed, as shown by the computing code they provide, the data analysis did consider ICS use as a time-dependent exposure, thus avoiding immortal time bias. Raymakers and colleagues used

As of 17 February, 2020, China has 70 635 confirmed cases of coronavirus disease 2019 (COVID-19), including 1772 deaths [1]. Human-to-human spread of the virus via respiratory droplets is currently considered to be the main route of transmission. However, exhaled air dispersion during non-invasive respiratory support may increase the risk of coronavirus transmission, and requires more attention from

Given the large public health burden of obstructive lung diseases, such as asthma and COPD, considerable research has been conducted to identify targeted interventional strategies, with nutrition and dietary intake receiving increasing amounts of attention. Diets rich in antioxidants and omega-3 fatty acids, and low in saturated fats and processed foods ( i.e. the Mediterranean diet) have been proposed

Interstitial lung diseases (ILDs) encompass a complex group of hundreds of lung disorders that affect lung tissue with variable morphologies and clinical presentations. The most extensively studied type of ILD is idiopathic pulmonary fibrosis (IPF), which is characterised by progressive pulmonary fibrosis, a decline in lung function, and high mortality with a histological pattern of usual interstitial

In the present issue of the European Respiratory Journal , Heerdt et al. [1] report on the validation of right ventricularejection fraction (RVEF) calculated from a pressure curve at cardiac catheterisation. This is of great interest as right ventricular (RV) function is the main determinant of symptomatology and outcome in pulmonary hypertension [2], and RVEF accordingly adds significantly to risk

Residual pulmonary impairment is common after treatment for tuberculosis (TB). Lung function data in patients with HIV-associated TB are scarce, especially in the context of paradoxical TB-associated immune reconstitution inflammatory syndrome (TB-IRIS) and prophylactic prednisone. We aimed to determine the prevalence of lung function abnormalities in patients with HIV-associated TB and CD4 counts

Evidence for associations between Mediterranean diet during pregnancy and childhood asthma, allergy and related outcomes is conflicting. Few cohorts have followed children to school age, and none have considered lung function. In the Avon Longitudinal Study of Parents and Children, we analysed associations between maternal Mediterranean diet score during pregnancy (estimated by a food frequency questionnaire