Primary ciliary dyskinesia (PCD), an inherited motile ciliopathy, is characterised by recurrent upper and lower respiratory tract infections, organ laterality defects, subfertility and neonatal respiratory distress due to impaired ciliary function.1 Over 50 PCD disease-causing genes have been identified that impact the structure and function of the cilia (figure 1, originally in Despotes et al 1).

Fibrosis is scarring due to the replacement of tissue architecture by extracellular matrix (ECM), which consists largely of collagen. Scarring progressively destroys organ structure and thereby impairs function. Accumulation of collagen is thought to be due to an increase in production, a reduction in degradation of collagen or a combination of both.1 If so, we might expect the ratio of production

Tuberculosis (TB) is a pressing global human health problem that resulted in over 10 million cases and 1.3 million deaths in 2022.1 The WHO proposes to end this global epidemic by 2035, which would necessitate reducing TB deaths by 95%, TB incidence by 90%, and eliminating catastrophic costs for TB-affected households compared with the 2015 levels. There is doubt this can be achieved. An established

Introduction While associations between ambient air pollution and respiratory health in chronic obstructive pulmonary disease (COPD) patients are well studied, little is known about individuals’ personal exposure to pollution and associated health effects by source. Aim To separate measured total personal exposure into indoor-generated and outdoor-generated pollution and use these improved metrics

Background Interstitial lung diseases (ILDs) include a large number of diseases associated with progressive pulmonary fibrosis (PPF), including idiopathic pulmonary fibrosis (IPF). Despite the rarity of each of the fibrotic ILDs individually, they cumulatively affect a considerable number of patients. PPF is characterised by an excessive collagen deposition leading to functional decline. Objectives

Introduction The pathogenesis of sarcoidosis involves tissue remodelling mediated by the accumulation of abnormal extracellular matrix, which is partly the result of an imbalance in collagen synthesis, cross-linking and degradation. During this process, collagen fragments or neoepitopes, are released into the circulation. The significance of these circulating collagen neoepitopes in sarcoidosis remains

Aims Spirometry is used by many clinicians to monitor asthma in children but relatively little is understood about its variability over time. The aim of this study was to determine the variability of forced expiratory volume in 1 s (FEV1) in children with symptomatically well-controlled asthma by applying three different methods of expressing change in FEV1 over 3-month intervals. Methods Data from

The mechanism of thrombocytopenia during acute pulmonary hypertension (PH) decompensation may be partly due to platelet aggregation in the lung. Platelet aggregates in explanted lung from 16 lung transplant patients during acute PH decompensation with and without thrombocytopenia were identified by immunohistochemistry. Scanning electron microscopy (SEM) was performed. 7 explant lung controls without

Tuberculosis (TB) control efforts are limited by ineffective characterisation of tuberculosis infection (TBI) —a heterogeneous spectrum of pre-clinical infection states, invisible to tools of routine clinical screening, that are associated with variable risk of progression to TB disease. In this prospective study, we use positron emission tomography-CT (PET-CT) as a high-resolution imaging modality

A 90-year-old man visited our hospital with a chief problem of haemoptysis lasting for a month. He had a medical history of bladder cancer, including transurethral resection, 10 years previously. Chest and abdominal CT demonstrated a large cystic lesion with thin walls surrounded by infiltration and ground-glass opacities in the left lower lobe of the lung (figure 1A), with no findings of bladder cancer

Background Childhood interstitial lung disease (chILD) encompasses a group of rare heterogeneous respiratory conditions associated with significant morbidity and mortality. Reports suggest that many patients diagnosed with chILD continue to have potentially progressive or fibrosing disease into adulthood. Over the last decade, the spectrum of conditions within chILD has widened substantially, with

Background Pulmonary microvasculature alterations are implicated in emphysema pathogenesis, but the association between pulmonary microvascular blood volume (PMBV) and emphysema has not been directly assessed at scale, and prior studies have used non-specific measures of emphysema. Methods The Multi-Ethnic Study of Atherosclerosis Lung Study invited participants recruited from the community without

An alarmingly high prevalence of tuberculosis (TB) was reported among the Saharia tribe in Madhya Pradesh, India. A community-based intervention study was undertaken to improve TB case finding during 2018–2021. The interventions mainly comprised active case detection through village TB volunteers using advocacy, communication and social mobilisation activities. A preintervention and postintervention

A 38-year-old woman was admitted for uterine fibroid surgery. A routine preoperative CT chest scan identified bilateral patchy, high-density shadows, with unclear boundaries, more evident in the upper lobes (figure 1A). She had no respiratory symptoms, such as coughing or expectoration, and was an office worker with allergies to seafood and mango. She denied having comorbidities including diabetes

Worldwide inequalities in care significantly impact respiratory health outcomes.1 2 Despite this, there are few studies examining how wheeze and asthma outcomes, including healthcare use and treatment decisions, vary with socioeconomic status and ethnicity in young children. Therefore, it is highly relevant to acknowledge the paper by D Lo et al , ‘Association between socioeconomic deprivation, ethnicity

Industry uses market segmentation of products to attract and retain consumers from a variety of groups based on demographics, interests, behavioural factors and common needs. This approach is used across many commercial sectors selling a diverse range of commodities including cleaning, beauty, food, automobile and electronic products. Consumers may be attracted to goods based on a number of characteristics

Interstitial lung disease (ILD) is a heterogeneous parenchymal disorder.1 2 Patients with ILD often present with non-specific symptoms such as a non-productive cough and exertional dyspnoea. The differential diagnosis for ILD is broad and includes conditions such as connective tissue disease (CTD), hypersensitivity pneumonitis, drug-associated ILD and granulomatous diseases. Furthermore, the initial

In sarcoidosis, any organ may be affected, and the majority of cases have pulmonary involvement. The disease course in pulmonary sarcoidosis varies considerably, from complete resolution to chronic disease and/or pulmonary fibrosis. The heterogeneity of sarcoidosis is a major challenge in treatment decision-making, and the hope is that subgrouping patients into phenotypes may help to provide the right

Objective People with advanced chronic obstructive pulmonary disease (COPD) have substantial palliative care needs, but uncertainty exists around appropriate identification of patients for palliative care referral. We conducted a Delphi study of international experts to identify consensus referral criteria for specialist outpatient palliative care for people with COPD. Methods Clinicians in the fields

Background Clinical studies have shown that glucagon-like peptide-1 receptor agonists (GLP-1 RA) can have beneficial effects on cardiopulmonary function. We conducted this longitudinal cohort study to compare the risk of cardiopulmonary outcomes and mortality between GLP-1 RA use and no use in patients with type 2 diabetes (T2D) and chronic obstructive pulmonary disease (COPD). Methods The study identified

Background Lung function testing remains a cornerstone in the assessment and management of interstitial lung disease (ILD) patients. The clinical implications of the Global Lung function Initiative (GLI) reference equations and the updated interpretation strategies remain uncertain. Methods Adult patients with ILD with baseline forced vital capacity (FVC) were included from the Australasian ILD registry

Introduction Given the heterogeneity of sarcoidosis, predicting disease course of patients remains a challenge. Our aim was to determine whether the 3-year change in pulmonary function differed between pulmonary function phenotypes and whether there were differential longitudinal changes by race and sex. Methods We identified individuals seen between 2005 and 2015 with a confirmed diagnosis of sarcoidosis

Objectives To examine the accuracy and impact of artificial intelligence (AI) software assistance in lung cancer screening using CT. Methods A systematic review of CE-marked, AI-based software for automated detection and analysis of nodules in CT lung cancer screening was conducted. Multiple databases including Medline, Embase and Cochrane CENTRAL were searched from 2012 to March 2023. Primary research

Background Preschool-aged children have among the highest burden of acute wheeze. We investigated differences in healthcare use, treatment and outcomes for recurrent wheeze/asthma in preschoolers from different ethno-socioeconomic backgrounds. Methods Retrospective cohort study using data from the Clinical Practice Research Datalink linked to Hospital Episode Statistics in England. We reported number

Introduction Cigarette smoking leads to altered DNA methylation at the aryl-hydrocarbon receptor repressor (AHRR) gene. However, it remains unknown whether pipe or cigar smoking is associated with AHRR methylation. We evaluated associations of non-cigarette tobacco use with AHRR methylation and determined if AHRR methylation was associated with smoking-related health outcomes. Methods Data were pooled

Objective Primary ciliary dyskinesia (PCD) severity has been related to genotype and levels of nasal nitric oxide (nNO). The most common TAS2R38 haplotypes (PAV/PAV, PAV/AVI, AVI/AVI) encoding the bitter taste receptor can affect nNO levels and thus could play a role in the susceptibility to respiratory infections. We assessed the impact of these polymorphisms on nNO production and Pseudomonas aeruginosa

Background and aims The recurrence of obstructive sleep apnoea (OSA) after positive airway pressure (PAP) therapy termination has physiological consequences that may increase cardiovascular (CV) risk. We aimed to determine whether PAP termination is associated with an increased incidence of major adverse CV events (MACE) compared with adherent PAP continuation. Methods Data from the Pays de la Loire

Mesenchymal stem cells (MSC) have immune regulatory properties that may ameliorate pathophysiological processes in sepsis. We determined the effect of allogeneic adipose-derived MSCs (Cx611) on the host response during sepsis due to community-acquired bacterial pneumonia (CABP) by measuring 29 plasma biomarkers and blood transcriptomes at six time points in 82 patients randomised to two intravenous

A 19-year-old female presented with a 6-month history of progressive shortness of breath. She reported a new onset of palpitations preceding her shortness of breath, which progressed progressively into dull chest pain and trepopnea, and she experienced dyspnoea while lying on her right side. With wheezing and rhinorrhoea, the symptoms became more prominent. She had no previous history of haemoptysis

A 73-year-old woman was found to have a pulmonary mass on a CT scan of the chest 10 years ago, but did not receive any treatment. Approximately 2 years ago, she started experiencing chest tightness and bloating, without other discomfort. These symptoms worsened half a month ago and were accompanied by dyspnoea. A CT scan (figure 1A,D) revealed a spherical mass in the left upper lobe lingular segment

A 47-year-old male presented with chronic cough, which has worsened over the last 5–6 months. Dyspnoea, fever and significant weight loss were denied. The patient had no history of smoking or vaping. The patient worked as an automotive technician in a car manufacturing plant specialised in aluminium-based vehicle bodies with frequent exposure to metallic fumes during polishing and welding of aluminium/beryllium

An elderly female, lifelong non-smoker, with rheumatoid arthritis treated with methotrexate and prednisone for 10 years was referred for evaluation of incidentally detected, randomly distributed pulmonary nodules on a CT performed for pleuritic chest pain and dyspnoea (figure 1A). Subsequent [18F]fluoro-d-glucose (FDG)-positron emission tomography (PET) showed the nodules had increased in size and

Background In adults and children with intellectual disability (ID), sleep -disordered breathing (SDB) is thought to be common. However, large epidemiological studies are lacking, and there are few studies on optimal methods of investigation and even fewer randomised, controlled intervention trials of treatment. Method Peer-reviewed publications from various databases were examined in line with search

The ACCESS trial ( Lancet Respir Med 2024;12(4):294–304) provides new evidence supporting the use of macrolides in community-acquired pneumonia (CAP). In this prospective, double-blind study, Giamarellos-Bourboulis and colleagues randomised patients admitted with CAP and receiving intravenous antibiotics to addition of either oral clarithromycin or placebo for 7 days. Participants from 18 Greek hospitals

Introduction Fluoroquinolones can cause severe collagen-associated adverse effects, potentially impacting the pulmonary connective tissue. We investigated the association between fluoroquinolones and spontaneous pneumothorax. Methods A case–time–control study was performed using the nationwide French reimbursement healthcare system database (SNDS). Cases were adults ≥18 years admitted for spontaneous

Sniff nasal inspiratory pressure (SNIP) is used to assess respiratory muscle strength in neuromuscular diseases like amyotrophic lateral sclerosis (ALS). The effect of contralateral nostril occlusion and mouth sealing on SNIP measurement are unclear. 81 participants were included (16 healthy, 39 patients with limb-onset ALS and 26 patients with bulbar-onset ALS). SNIP was obtained with combinations

Silicosis is the most ancient form of occupational lung disease, being known to have caused disability and the premature death of workers for many millennia.1 Despite this, silicosis continues to be a global health problem,2 accounting for almost 13 000 deaths worldwide in 2019.3 Those at risk of this disease commonly work in industries that involve either cutting through silica-containing material

In 2019, a record 4.5 billion passengers travelled by air.1 This fell markedly during the COVID-19 pandemic, but in 2023, the industry’s measure of overall airline ‘traffic’, revenue passenger kilometres, was back to 94% of the 2019 figures, with predictions that 2024 will see new record passenger numbers (see figure 1).2 This highlights how common air travel is and reinforces the idea that it is highly

Over the last few decades, women’s health has slowly shifted closer to the forefront of research.1 There have been multiple major campaigns at academic, charity and government level, for the improvement of women’s health globally. In 2021, the UK government implemented the Women’s Health Strategy to improve the health of women in the UK over 10 years and Asthma and Lung UK published their report on

The cystic fibrosis transmembrane conductance regulator (CFTR) modulator compound, ivacaftor, reached its 10-year anniversary of US Food and Drug Administration (FDA) approval in 2022. This long-standing experience captured within US CF foundation patient registry (US CFFPR) data up to December 2019 was harnessed by Merlo and colleagues in this edition of the journal to provide further insight into

Asthma is the most prevalent chronic disease of childhood. Based on the Global Burden of Diseases, Injuries and Risk Factors Study, the global incident cases of asthma in children increased by 7.07% from 1990 to 2019, the highest incidence being observed in children under 5 years old.1 Among these youngest children, asthma is difficult to diagnose, and is often defined by wheezing episodes characterised

Introduction Asthma is an inflammatory airways disease encompassing multiple phenotypes and endotypes. Several studies suggested gene expression in nasal epithelium to serve as a proxy for bronchial epithelium, being a non-invasive approach to investigate lung diseases. We hypothesised that molecular differences in upper airway epithelium reflect asthma-associated differences in the lower airways and

Background Ivacaftor (IVA) improves lung function and other extrapulmonary outcomes in people with cystic fibrosis (CF). However, the effect of initiating IVA at earlier versus later ages has not been studied. Methods We conducted an observational cohort study of people in the US CF Foundation Patient Registry aged ≥6 years with ≥1 CF transmembrane conductance regulator–gating mutation to compare the

Background Ivacaftor (IVA) has been shown to improve lung function and other clinical outcomes in people with cystic fibrosis (CF). A decade of real-world IVA availability has enabled the examination of long-term outcomes with this treatment. This retrospective, longitudinal cohort study investigated the impact of IVA on mortality rate and health outcomes among people with CF in the US. Methods Data

Background Silicosis, a chronic respiratory disease caused by crystalline silica exposure, is a persistent global lung health issue. No systematic review of the relationship between cumulative respirable crystalline silica (RCS) exposure and silicosis exists. UK exposure limits are currently under review. We therefore performed a systematic review and dose-response meta-analysis of this relationship

Background Infections in childhood remain a leading global cause of child mortality and environmental exposures seem crucial. We investigated whether urbanicity at birth was associated with the risk of infections and explored underlying mechanisms. Methods Children (n=633) from the COPSAC2010 mother–child cohort were monitored daily with symptom diaries of infection episodes during the first 3 years

Background Antenatal factors and environmental exposures contribute to recurrent wheezing in early childhood. Aim To identify antenatal and environmental factors associated with recurrent wheezing in children from birth to 48 months in the mother and child in the environment cohort, using time-to-event analysis. Method Maternal interviews were administered during pregnancy and postnatally and children

Rationale Early natural menopause (early-M; <45 years of age) increases the risk of lung morbidities and mortalities in smokers. However, it is largely unknown whether early-M due to surgery demonstrates similar effects and whether menopausal hormone therapy (MHT) is protective against lung diseases. Objectives To assess the associations of early-M and MHT with lung morbidities and mortalities using

Background Passengers on long-haul flights frequently consume alcohol. Inflight sleep exacerbates the fall in blood oxygen saturation (SpO2) caused by the decreased oxygen partial pressure in the cabin. We investigated the combined influence of alcohol and hypobaric hypoxia on sleep, SpO2 and heart rate. Methods Two groups of healthy individuals spent either two nights with a 4-hour sleep opportunity

Silicosis due to artificial stone (AS) has emerged over the last decade as an increasing global issue. We report the first eight UK cases. All were men; median age was 34 years (range 27–56) and median stone dust exposure was 12.5 years (range 4–40) but in 4 cases was 4–8 years. One is deceased; two were referred for lung transplant assessment. All cases were dry cutting and polishing AS worktops with

We quantified the proportion of diagnoses of pulmonary fibrosis (PF) among 25 136 people with lung cancer and 250 583 matched controls and compared the natural history of lung cancer in people with and without PF. Diagnoses of PF were more common in people with lung cancer than those without (1.5% vs 0.8%, OR 1.97; 95% CI 1.77 to 2.21). Within people with PF, squamous cell carcinoma was more (22.9% vs

A 38-year-old man with underlying transfusion-dependent beta-thalassemia and a history of splenectomy in August 2023 presented in December of the same year, complaining of one month of dyspnoea and orthopnoea. The chest radiograph revealed bilateral pleural effusion, with a left-sided predominance. Diagnostic and therapeutic thoracentesis demonstrated straw-coloured serous pleural fluid (pf) with an

Rationale/Objectives Despite plausible pathophysiological mechanisms, research is needed to confirm the relationship between sleep, circadian rhythm and delirium in patients admitted to the intensive care unit (ICU). The objective of this review is to summarise existing studies promoting, in whole or in part, the normalisation of sleep and circadian biology and their impact on the incidence, prevalence

Household air pollution has been estimated to be responsible for 3.2 million preventable deaths every year globally. With biomass exposure and environmental pollution linked to exacerbations of airways disease, this health impact disproportionately affects low and middle income countries. Puzzolo et al ( Lancet Resp Med 2024;12(4):281–293) undertook a systematic review and included 116 studies in the

A 48-year-old man, non-smoker, with no known comorbidities, presented with shortness of breath, cough with mucoid expectoration, loss of appetite and loss of weight for 6 weeks. There was no history of fever, hemoptysis or wheezing. He had received multiple courses of antibiotics from elsewhere before presenting to our centre with no relief of symptoms. On examination, crepitations were audible in

A 44-year-old woman presented with a persistent diffuse skin rash for 3 months, along with progressively worsening dyspnoea, cough and fatigue over the past 2 weeks. Laboratory results and physical examination were unremarkable, except for decreased breath sounds over the right lung and multiple palpable nodular skin lesions over the chest, abdomen and limbs (figure 1). Chest radiograph revealed a

Tests based on polymerase chain reactions are increasingly important in the microbiological diagnosis of tuberculosis (TB). Park et al1 have extended the use of Xpert Ultra to include material obtained at bronchoscopy, and particularly its use for samples obtained by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA). In addition, immediate cytology with rapid onsite evaluation

Quantification of the physiological changes in people with cystic fibrosis (pwCF) has long been important for clinical diagnosis. Since the original discovery of abnormal sweat electrolytes in pwCF, the standardised pilocarpine iontophoresis sweat test was developed1 to clearly distinguish those with CF. While the sweat chloride test remains an important early step in the diagnostic algorithm, complementary

The task is clear: we need better treatments for pulmonary fibrosis (PF). The two antifibrotic therapies licensed for idiopathic pulmonary fibrosis (IPF) have shown efficacy in other forms of PF but do not meet the needs of people living with PF; drugs that can halt or even reverse progression and, importantly, improve quality of life. Although a number of new potential therapies are now reaching late-stage

Organic dusts are mixtures of particles originating from plants, animals and often endotoxins from gram-negative bacteria. Several occupations, including farming (where exposures can occur during grain, animal handling and feeding), woodworking (where exposures can occur from wood dust) and textile work (during cotton processing), are the primary sources of occupational exposure to organic dust.1 2