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  • Journal article
    Saralaya D, Mustapa MN, Ferreira J, Kocks JWH, Brailsford W, Rosengren S, Spata E, Belvisi MG, Leander J, Riff C, De Palo G, Windgassen D, Chalmers JD, Russell REK, Hurst JR, Śmiałowski A, Hughes Ret al., 2026,

    A phase 2a, double-blind, randomised, placebo-controlled trial of the myeloperoxidase inhibitor, mitiperstat, in participants with chronic obstructive pulmonary disease.

    , Eur Respir J

    BACKGROUND: Neutrophilic inflammation is a common feature of chronic obstructive pulmonary disease (COPD). Mitiperstat, an inhibitor of myeloperoxidase expressed in neutrophils, may have potential as a COPD treatment. METHODS: This phase 2a, randomised, placebo-controlled, double-blind, parallel-arm, event-driven trial evaluated the efficacy and safety of mitiperstat 5 mg daily up to 24 weeks (NCT05492877). Adults (40-80 years) with moderate-to-severe COPD, at high risk of exacerbation, and receiving dual or triple inhaled therapy were included. The primary endpoint was time to first composite endpoint for exacerbations in COPD (COPDCompEx) event. Secondary endpoints included time to first moderate-to-severe COPD exacerbation, post-bronchodilator forced expiratory volume in 1 s (post-BD FEV1) change at Week 12, respiratory symptoms, disease impact and safety. RESULTS: Overall, 381 participants (mean age, 66.0 years; 39.6% female) were randomised to mitiperstat (n=189) or placebo (n=192). In total, 125 (66.1%) mitiperstat-treated versus 125 (65.1%) placebo-treated participants experienced COPDCompEx events over 24 weeks. There was no improvement in time to first COPDCompEx event (hazard ratio [HR] 1.07 [90% confidence interval (CI) 0.87, 1.32]; p=0.599) or time to first moderate-to-severe COPD exacerbation (HR 1.23 [90% CI 0.88, 1.73]) with mitiperstat versus placebo. Improvements in post-BD FEV1, respiratory symptoms or disease impact were not seen with mitiperstat. A similar proportion of participants in both groups reported adverse events; seven mitiperstat-treated participants and two placebo-treated participants had pneumonia during the study. CONCLUSION: These results indicate that the risks outweigh the benefits of mitiperstat as a treatment for COPD.

  • Journal article
    Knox-Brown B, Sylvester K, Amaral A, 2026,

    Physiological quotients for discriminating mortality and respiratory outcomes: a UK biobank cohort study

    , ERJ Open Research, ISSN: 2312-0541

    BackgroundSpirometry is traditionally interpreted using reference-based metrics derived from equations such as those from the Global Lung Function Initiative (GLI). Physiological quotients, which express lung function relative to a lower physiological boundary, have been proposed as an alternative approach. We aimed to compare the prognostic performance of physiological quotients with conventional reference-based metrics including both the GLI 2012 ethnicity-specific and the GLI 2023 Global equations, in a large population-based cohort.MethodsWe analysed data from 270,599 UK Biobank participants aged 40–69 years with baseline spirometry. Physiological quotients were calculated for FEV₁, FVC, and FEV₁/FVC using previously established first percentile boundaries. Associations with all-cause and cause-specific mortality, respiratory hospitalisation, respiratory symptoms, and self-reported respiratory diagnoses were examined using Cox proportional hazards and logistic regression models.ResultsOver a median follow-up of 15.7 years, 26,197 (10%) participants died. Lower physiological quotients were consistently associated with adverse outcomes. Each 1-SD decrement in FEV₁Q, FVCQ, and FEV₁/FVCQ was associated with higher all-cause mortality (HRs 1.06–1.09), cardiovascular mortality (HRs 1.06–1.20), respiratory mortality (HRs 1.28–1.77), and respiratory hospitalisation (HRs 1.04–1.41). Discrimination for all-cause mortality was modest (C-statistics 0.64–0.65), moderate for cardiovascular mortality (0.72–0.73), and good for respiratory mortality (0.76–0.79). Discrimination of respiratory hospitalisation was modest but slightly higher for FEV₁Q and FVCQ (0.65) than for percent predicted values and z-scores (0.61–0.62). Across mortality outcomes, discriminative performance was similar between physiological quotients, percent predicted values, and z-scores. ConclusionPhysiological quotients demonstrated prognostic performanc

  • Journal article
    Pates K, Premuda C, Frost F, Finney L, Nagar J, Bhattacharyya A, Dhar R, Aboelhassan A, Ru C, Thornton C, Tryfon S, Papadopoulou E, Dřevínek P, Kwok WC, Ukor E-F, Blasi F, Thee S, Ekkelenkamp M, Shah A, Gramegna Aet al., 2026,

    PRESIDE protocol: a global registry of antimicrobial resistance in chronic lung disease

    , ERJ Open Research, ISSN: 2312-0541

    Antimicrobial resistance (AMR) is an escalating global health threat. Chronic lung diseases (CLDs) represent a key area in which AMR poses unique and often underestimated challenges, yet high-quality epidemiological data remain scarce. To address this gap, the European Respiratory Society Clinical Research Collaboration on AMR in Lung Infections (AMR-Lung CRC) has developed PRESIDE, an international prospective observational registry designed to evaluate the prevalence and burden of AMR in patients with CLD. PRESIDE is a global, multicentre study recruiting paediatrics and adults with CLD who have undergone respiratory microbiological testing as part of routine clinical care within the year prior to data collection. Participating centres enroll patients during two-week recruitment periods, held twice yearly over five years from 2025 to 2030. The registry captures a comprehensive set of core variables, including demographics and smoking history, CLD type and aetiology, microbiological history, comorbidities, clinical characteristics, therapeutic exposures, and respiratory microbiology results.

  • Journal article
    Wedzicha JA, Finney LJ, Martinez FJ, 2026,

    Every exacerbation counts: shifting the dial for future exacerbation risk.

    , Am J Respir Crit Care Med, Vol: 212, Pages: 1675-1676
  • Journal article
    Burney P, Knox-Brown B, Amaral A, 2026,

    The use of spirometry to define airflow obstruction and diagnose COPD

    , European Respiratory Journal, Vol: 68, ISSN: 0903-1936

    The recent statement from GOLD/GLI on the use of spirometry is insufficient, particularly in the global South.

  • Journal article
    Canizales J, Schofield S, Shamji MH, Cullinan P, Jones M, Feary Jet al., 2026,

    Patterns of mouse allergen–specific IgE and IgG4 in contemporary animal research environments

    , Clinical and Experimental Allergy, Vol: 56, Pages: 983-985, ISSN: 0954-7894
  • Journal article
    Dvorscek AR, Kushnir AS, Dibben O, Kijak GH, Thwaites RSet al., 2026,

    Mucosal immunity as a vaccine-induced correlate of protection against influenza.

    , Mucosal Immunol, Vol: 19

    Licensure of influenza vaccines relies on serum hemagglutination inhibition (HAI) titers, a correlate of protection (CoP) that was developed more than 50 years ago and which is only poorly predictive of protection. This is especially true of immunity induced by intranasal live attenuated influenza vaccines (LAIVs). Unlike intramuscular inactivated influenza vaccines (IIVs), LAIV and natural infection selectively stimulate mucosal immunity. Developments in mucosal immunology now enable measurement of diverse aspects of mucosal immunity, including the frequencies and functions of nasal antibodies, T cells, and B cells. This review assesses the potential of new sampling and assay approaches that may overcome inconsistent findings from previous methodologies. Standardization of the collection and assessment of mucosal samples is essential in developing new CoPs for vaccine development and licensure of novel vaccines that induce nasal protection and are more effective in prevention of viral transmission.

  • Journal article
    Quintero Santofimio V, Ma J, Potts J, Kromhout H, Feary J, Janson C, Svartengren M, Anand M, Jogi R, Gislason T, Juvekar S, Nielsen R, Erhabor G, Harrabi I, Devereux G, Malinovschi A, Agarwal D, Ahmed R, Garcia-Larsen V, Nafees A, Burney P, Amaral A, On behalf of the BOLD Collaborative Research Groupet al., 2026,

    Association of respiratory health with occupational exposures in the Burden of Obstructive Lung Disease (BOLD) cohort: a multinational longitudinal study

    , BMJ Open Respiratory Research, Vol: 31, ISSN: 2052-4439

    Background: Occupational exposures are contributors to chronic respiratory diseases, particularly in low- and middle-income countries (LMICs), where regulatory policies are limited. We investigated associations between occupational exposures and respiratory outcomes using longitudinal data from the Burden of Obstructive Lung Disease study.Methods: We analysed data from 4,237 participants across 17 sites, mostly in LMICs. Occupational exposures were assessed by self-report (never vs ever) and the ALOHA+ Job Exposure Matrix for vapours, gases, dusts, fumes (VGDF), pesticides, solvents, and metals (cumulative exposure). Respiratory outcomes included: forced expiratory volume-in-1-second (FEV₁), forced vital capacity (FVC), the FEV₁/FVC, and respiratory symptoms. Associations were examined using multilevel linear and logistic regression models adjusted for age, sex, smoking, pack-years, education, body mass index, and baseline FVC. We further explored sex differences and non-linear relationships for symptoms.Results: Over a median 10 years of follow-up, FEV₁/FVC decline was associated with moderate (β=–1.34; 95%CI: –2.32, –0.35) and high (β=–1.79; 95%CI: –3.33, –0.20) exposure to VGDF and low (β=–1.11; 95%CI: –2.11, –0.08) and high (β =–2.16; 95%CI: –4.08, –0.24) exposure to pesticides. Increased risk of wheeze was associated with moderate (RR=1.45; 95%CI: 1.05-2.15) and high (RR=1.89; 95%CI: 1.100-3.26) exposure to pesticides. Associations did not differ by sex. There was weak evidence of non-linear exposure–response relationships, and no associations with solvents or metals.Conclusions: A significant decline in FEV1/FVC was associated with exposure to VGDF and pesticides. Increased risk wheeze was also associated with exposure to pesticides. These findings underscore the need for continued monitoring in high-exposure settings, particularly in LMICs.

  • Journal article
    Meghji J, Engel N, Kagima W, Kinya I, Kionga Y, Lesosky M, Liyoyo A, Madan J, Mpagama S, Obase Ehrim J, Ogundare A, Ozoh O, Orina F, Parker Allen J, Paul EK, Potts J, Tuwabunze L, Yates N, Chakaya Muhwa Jet al., 2026,

    The ITARA research programme: investigating Integrated Tuberculosis and Respiratory care in Africa using transdisciplinary methods

    , BMJ Open, Vol: 16, ISSN: 2044-6055

    Introduction Pulmonary tuberculosis (PTB) and chronic respiratory diseases (CRDs) are closely linked. Affected groups present with similar symptoms and share many risk factors (eg, poverty-related factors, smoking, occupational exposures). PTB is itself an independent risk factor for chronic lung disease. However, in many high TB-incidence settings health services for these conditions are provided separately, with little integration of prevention, diagnosis or care.Methods and analysis We describe a transdisciplinary programme of research investigating strategies for integrated TB-CRD care in Arusha, Tanzania, Nairobi, Kenya and Lagos, Nigeria, using clinical, health economic, health systems and qualitative research methods. A prospective clinical cohort study will describe the burden and impact of non-TB respiratory disease (eg, asthma, chronic obstructive pulmonary disease, post-TB lung disease) among adolescents and adults presenting to primary and secondary health facilities with chronic cough who would normally be managed via TB care pathways. Health economics methods will explore patient costs of non-TB respiratory disease, facility-level costs of integrated TB/respiratory diagnostics and will develop a modelling framework to estimate the costs and consequences of integration more broadly. In-depth interviews, focus group discussions, observations and participatory methods will be used to explore lived experiences of chronic respiratory symptoms, disease and exposures among patients and providers, and to identify and address challenges around respiratory health and care. Lastly, existing TB and CRD healthcare services and systems in our three research sites will be described, and local, national and policy level understandings of ‘integration’ of TB and CRD care will be explored. Together, the findings of this work will be used to develop context-informed model(s) of integrated TB-CRD care and a theory of change and framework for evaluation in futu

  • Journal article
    Bisson GP, Allwood B, Byrne A, Günther G, Khosa C, Navuluri N, Nightingale R, Schoeman I, van der Zalm MM, Meghji J, Auld Set al., 2026,

    Post-tuberculosis lung disease: a case definition for use in research studies.

    , Lancet Infect Dis, Vol: 26, Pages: e315-e325

    Despite growing awareness of the substantial burden of long-term pulmonary impairment among tuberculosis survivors, marked variability in how post-tuberculosis lung disease is defined across research studies limits the comparison of findings and synthesis of evidence. To facilitate greater harmonisation within the field, we propose a case definition for post-tuberculosis lung disease for use in research studies. Conceptual aspects of this case definition were initially developed with input from a broad group of stakeholders at the 2nd International Post-Tuberculosis Symposium and were refined by the authors after the Symposium. Guiding principles for the definition include specificity, feasibility in settings with high tuberculosis disease burdens, probable relevance to long-term health outcomes, and applicability across the lifespan. The definition is designed to be used alongside, rather than instead of, study-specific definitions used to explore primary study hypotheses, and is accompanied by a reporting framework. The case definition has three components: that the individual had previous pulmonary or pleural tuberculosis disease and does not have tuberculosis disease at the time of evaluation; that the individual has, at the time of assessment, evidence of pulmonary disease with abnormalities in at least two of three clinical domains of lung function, respiratory symptoms, and chest imaging; and that the pulmonary disease manifestations should be attributable at least in part to previous tuberculosis disease. This definition is developed in the absence of data on long-term patient outcomes and will need to evolve over time in response to emerging evidence. However, we believe this proposed definition will lead to greater consistency and rigor across studies of post-tuberculosis lung disease with the goal of improving care and quality of life for millions of tuberculosis survivors worldwide.

  • Journal article
    De Boeck K, Burgel P-R, Bierlaagh M, Hill K, Amaral M, Birimberg-Schwartz L, Brewington JJ, Davies JC, Karadag B, Martin U, Mense M, Pedemonte N, Sharma N, Tayor-Cousar JL, Sermet I, Beekman JMet al., 2026,

    ECFS statement on theratyping and theranostics in the context of rare and ultrarare CFTR variants in people with CF.

    , J Cyst Fibros, Vol: 25, Pages: 584-593

    Genotype-based drug development has yielded highly effective therapies, notably the triple combinations elexacaftor/tezacaftor/ivacaftor (ETI) and vanzacaftor/tezacaftor/deutivacaftor (VTD), now approved in Europe for people with CF having at least one non-class I variant. However not all these people with CF will respond to ETI or VTD, and a few not under the label may respond. Facilitating opportunities to access for patients with rare variants has required a shift in paradigm toward functional testing-based access. This approach assesses the potential benefit of modulator therapy using in vitro functional assays, either in engineered systems expressing defined CFTR variants (theratyping) or in patient-derived tissues (theranostics). We review theranostics as a critical tool for personalized medicine in CF, highlighting its validation in in vitro models derived from patients' own cells such as human intestinal organoids and human nasal epithelial cells. We discuss the current regulatory landscape regarding modulator approval and propose strategies for improving equitable access to effective treatments for all people with CF. Importantly, we advocate for functional assays to be accepted as standalone evidence of drug efficacy for patients with rare variants. Theranostic approaches remain critical when theratyping has not been achieved, and genetic data is not available or clearly interpretable. Indeed, theranostics has emerged as an essential pillar of CF drug access, complementing genotype-driven strategies. As the field advances, continued validation, standardization, and regulatory integration of in vitro functional assays will be key to ensuring that every person with CF-regardless of their genotype-has the opportunity to benefit from precision therapies.

  • Journal article
    Davies JC, Bakkeheim E, Chansard A, Drevinek P, Dubois C, Matthews J, Sheremet M, Smith Cet al., 2026,

    Perspective of the European Cystic Fibrosis Society on Improving Global Cystic Fibrosis Care.

    , Pediatr Pulmonol, Vol: 61

    INTRODUCTION: Outcomes for people with the inherited disease, cystic fibrosis, have improved greatly over the last few decades, but one result of this is a widening gap between regions with high income and well-resourced healthcare systems and low/middle income countries. The gap stretches from newborn screening programs, provision of standard diagnostics and genetic testing through to access to standard of care therapies. METHODS AND RESULTS: This paper describes the various initiatives of the European Cystic Fibrosis Society: our Patient Registry, a Twinning Program linking centers from different regions and our Educational Program. CONCLUSIONS: The European Cystic Fibrosis Society recognizes this as a major issue and seeks through these programs to support colleagues, patients and families in low/middle income countries.

  • Journal article
    Meme H, Matu S, Orina F, Miheso B, Kiplimo R, Ndombi A, Kathure I, Kinyanjui J, Amukoye E, Chakaya J, He N, Bowyer C, Gray CM, Lesosky M, Mortimer K, Semple S, West SE, Zurba L, Binegdie AB, El Sony A, Devereux Get al., 2026,

    Lung function across the life course in Kenya: a series of cross-sectional surveys.

    , ERJ Open Res, Vol: 12, ISSN: 2312-0541

    BACKGROUND: The high prevalence of COPD in sub-Saharan Africa is poorly understood. In high-income countries, COPD is the consequence of suboptimal lung growth during childhood and/or accelerated lung function decline in adult life. We have conducted cross-sectional studies to measure the lung function of children and adults in Kenya and to identify associations with age. METHODS: We performed spirometry in three groups in Kenya: a random sample of schoolchildren in two districts of urban Nairobi and age/sex-stratified representative community samples of adults in Nairobi and rural Machakos. Forced expiratory volume in 1 s (FEV1) and forced vital capacity were expressed as z-scores using race-neutral GLI-Global reference equations. RESULTS: The mean (95% CI) FEV1 z-score in Nairobi schoolchildren (n=2373, median age 10 years (IQR 8-13) 52% girls) was -0.60 (-0.64- -0.55); in Nairobi adults (n=2936, median age 32 years (24-43), 62% female) -0.49 (-0.53- -0.45); and in Machakos adults (n=1607, median age 46 years (35-59), 65% female) -0.67 (-0.72- -0.61). In adults, FEV1 was negatively associated with age (FEV1 z-score regression coefficient β -0.005/year (95% CI -0.009- -0.002) p=0.005, and there was a negative interaction between residence in Nairobi and age, β -0.006/year (95% CI -0.011- -0.001), p=0.020. CONCLUSION: The lung function of children and adults in Kenya was lower than predicted by race-neutral Global Lung Function Initiative (GLI)-Global reference equations. In adults, a negative association between lung function and age was greater in urban, than in rural, settings. Further work is required to identify and mitigate relevant influences.

  • Journal article
    Gandhi SA, Liu GY, Fazio JC, Amubieya O, Barnes H, Cavalin C, Cohen RA, Fireman E, Garcia SR, Harrison RJ, Houlroyd JL, Hua JT, Jones CM, Mao L, Ramkissoon C, Schenker M, Feary J, Harvey RR, Menéndez-Navarro A, Nemery B, Redlich CA, Reynolds CJ, Hoy RF, Cummings KJ, American Thoracic Society Assembly on Environmental, Occupational and Population Healthet al., 2026,

    Silicosis in the Artificial Stone Countertop Industry: An Official American Thoracic Society Workshop Report.

    , Ann Am Thorac Soc

    Artificial stone-associated silicosis (AS silicosis) has emerged over the past decade as a severe, rapidly progressive, and preventable occupational lung disease affecting workers who manufacture, fabricate, and install artificial stone countertops. Characterized by short latency, accelerated progression, and high morbidity and mortality, AS silicosis disproportionately affects young workers employed in precarious conditions. In response to the growing global burden of disease, this American Thoracic Society workshop was convened in 2025 to review the current state of knowledge regarding AS silicosis, synthesize the current evidence, and identify priorities for research, clinical care, public health surveillance, and prevention. Workshop participants reviewed data spanning exposure science, epidemiology, clinical manifestations, health equity, and policy responses. Evidence demonstrates that artificial stone (AS) dust is highly toxic, containing high concentrations of respirable crystalline silica, resin-derived volatile compounds, and trace metals, resulting in exposures that routinely exceed occupational exposure limits. Despite widespread implementation of wet methods, ventilation, and respiratory protection, hazardous exposures persist across diverse settings globally, highlighting fundamental limitations of existing control strategies. Clinically, AS silicosis is associated with high rates of progressive massive fibrosis, autoimmune disease, infection, respiratory failure, and increasing need for lung transplantation. Treatment options remain limited, underscoring the importance of early detection and exposure cessation. The workshop identified critical gaps in medical screening and public health surveillance worldwide, with inconsistent regulatory frameworks, low compliance, underreporting, and delayed diagnoses. Case detection is often dependent on symptomatic presentation rather than proactive screening, exacerbating disease severity and inequities in care.

  • Journal article
    Sinadinos A, Bell R, Juarez-Molina CI, Meng C, Castells E, Viegas MA, Gill DR, Hyde SC, Griesenbach U, Alton EWFWet al., 2026,

    Using the nose as a factory to secrete proteins into the lungs or circulation

    , Molecular Therapy Advances, Vol: 34, ISSN: 3117-387X

    Targeting of the nasal epithelium for sustained therapeutic protein secretion represents a potential non-invasive lentiviral vector application strategy. Using reporter imaging, molecular, and radiopharmaceutical tracing methods in mice, we have developed an intranasal (nose-only) dosing strategy with a Sendai-virus envelope glycoprotein pseudotyped lentiviral vector (rSIV.F/HN). Using multiple (up to 10) small volume (5 μL) intranasal bolus applications, technetium radiotracer showed >90% liquid retention in the murine head and <1% in the lung. Following vector administration, transgene expression was dose-related in the nose with minimal lung expression. No acute nasal toxicity was associated with nose-only delivery. Next, we compared levels of a secreted protein, Gaussia luciferase (Gluc), in the airways and serum after nose-only and intravenous administration of rSIV.F/HN-Gluc (2e8 TU/mouse). Gluc expression in the nose and lungs was higher following nose-only versus intravenous administration. Serum levels were similar after either route of administration. Finally, nose-only delivery of rSIV.F/HN encoding GM-CSF, led to sufficient lung levels of this therapeutic protein to correct disease biomarkers in a mouse model of pulmonary alveolar proteinosis. We conclude that non-invasive administration of a lentiviral vector to the nasal epithelium provides a safe and convenient route for secreted protein production and is readily translatable into humans.

  • Journal article
    Nabisere A, Wakasiaka S, Dodd J, Byrne-Davis L, Lesosky M, Lavender T, Mills TA, Ayebare Eet al., 2026,

    Translation and evaluation of the Perinatal Grief Intensity Scale in Kenya and Uganda.

    , BMC Pregnancy Childbirth, Vol: 26

    BACKGROUND AND AIM: Stillbirth or neonatal death are distressing life experiences for parents. In many sub-Saharan African countries, bereavement care and support are inadequate. Context-appropriate interventions are needed to improve perinatal bereavement care. To assess whether these interventions work, reliable assessment tools are required. This study aimed to translate and evaluate the Perinatal Grief Intensity Scale [PGIS] for use among bereaved women in Kenya and Uganda. METHODS: A mixed-methods study, involving cross cultural translation, adaptation and psychometric evaluation in Kenya and Uganda. The PGIS was translated into Kiswahili and Luganda with input from community engagement groups and stakeholders. Pilot-testing was conducted in 2 stages; 'Think aloud' interviews (n = 16) and test-retest (n = 20). The finalised tools were tested with 160 women who had experienced stillbirth or neonatal death, 20 in the test-retest phase and 140 in wider testing. Analyses included test-retest reliability, internal consistency and exploratory associations with participant characteristics using single variable linear regression. RESULTS: Agreed translations of the PGIS in Kiswahili and Luganda were produced. In pilot testing, test-retest consistency was high with an intraclass correlation coefficient of 0.840 (95% CI, 0.590-0.937). Wider testing demonstrated acceptable internal consistency, Cronbach's alpha of the total PGIS score was 0.737, confirming overall reliability, with similar results in the pilot testing and wider testing subgroups. Single variable linear regression showed that PGIS scores were higher for women in Uganda (coefficient 0.123, 95% Confidence Interval (CI) 0.042 to 0.205, p value = 0.003) compared to Kenya, and higher for caesarean births (coefficient 0.113, 95% CI 0.014 to 0.211, p value = 0.025) compared to vaginal births. CONCLUSION: Preliminary evaluation of the Kiswahili and Luganda ve

  • Journal article
    Short C, Semple T, Abkir M, Efthyvoulou C, Padley S, Rosenthal M, McNally P, Tiddens H, Caudri D, Tibiletti M, Parker GJ, Davies JCet al., 2026,

    Oxygen-enhanced MRI and multiple breath washout with Short extension reveal cystic fibrosis lung disease progression despite triple modulator therapy.

    , Thorax

    INTRODUCTION: The current cystic fibrosis (CF) care era, while hugely welcome, raises new challenges, particularly the need for more sensitive pulmonary outcome measures. Seeking further optimisation, we previously developed a Short extension to multiple breath washout measure (MBWShX) which captures previously overlooked, under-ventilated lung units but lacks regional information. Functional lung MRI addresses this limitation. We hypothesised these measures would be more sensitive to change in tracking CF lung disease than usual clinical respiratory function tests. METHODS: Forty-six people with (pw)CF, median age 15 (range 6-55) years were recruited to a single-centre study. While clinically stable, pwCF performed OE-MRI, MBW+/-ShX and spirometry at baseline and at 6 monthly intervals over 18 months of follow-up. A subgroup of pwCF (n=20) and age-matched healthy controls (HC, n=20) performed two repeatability visits within 6 weeks. RESULTS: OE-MRI/MBWShX were well tolerated, differentiated HC and CF groups, and were repeatable with negligible differences between two visits <6 weeks apart. OE-MRI/MBWShX parameters worsened at 12 months (p<0.05) and 18 months (p<0.01). In contrast, conventional measures of pulmonary function (FEV1+ LCI2.5) did not change significantly. CONCLUSIONS: OE-MRI/MBWShX are novel, sensitive tools to track progression of abnormalities in lung structure/function. Such progression may not be detected by conventional outcome measures. CF transmembrane conductance regulator (CFTR) modulators have been transformative for many pwCF and generally lead to substantial improvements in lung health. Stable FEV1 over longer time periods and, during mucoactive treatment withdrawal, may give false reassurance. OE-MRI/MBWShX reveal the likely less welcome reality that lung-disease progresses despite CFTR modulators. These measures could be considered in future studies when enhanced sensitivity is required.

  • Journal article
    Harker JA, Thwaites RS, 2026,

    Restoring β2AR responsiveness in neonatal RSV.

    , Am J Respir Crit Care Med, Vol: 212, Pages: 1189-1191
  • Journal article
    MacLeod MA, Knott KD, Nicol ED, Wedzicha JAet al., 2026,

    Reply to Liu et al.: Considerations on stratified analysis and biomarker use in coronary artery disease detection in chronic obstructive pulmonary disease.

    , Am J Respir Crit Care Med, Vol: 212, Pages: 1368-1369
  • Journal article
    Burney P, Bagkeris E, Potts J, Nielsen R, Devereux G, El Rhazi K, Aquart-Stewart A, Nafees A, Koul P, Mahesh P, Agarwal D, Juvekar S, Gislason T, Denguezli M, Harrabi I, Paraguas S, Jogi R, Erhabor G, Buist AS, Janson C, Amaral A, BOLD Collaborative Research Groupet al., 2026,

    A cohort study of forced vital capacity, airway obstruction and survival in the multinational Burden of Obstructive Lung Disease (BOLD) study

    , International Journal of Epidemiology, Vol: 55, ISSN: 0300-5771

    BackgroundIn the USA, higher forced vital capacity (FVC) is linked with longer survival, and FVC is associated with survival independently of ethnicity. The implications for the low FVC values in parts of Asia and Africa are unknown.MethodsWe used data from 16 sites of the multinational Burden of Obstructive Lung Disease (BOLD) study that completed follow-up of participants between 2019 and 2021 and reported at least five deaths between baseline and follow-up. We assessed the association between mortality and FVC and one-second Forced Expiratory Volume (FEV1)/FVC ratio within each site using Cox proportional hazards models. These models were adjusted for age, smoking, height and weight. Effect estimates from all sites were combined using meta-analysis. Systematic regional differences were investigated.ResultsOf 9,927 study participants with follow-up data, 1,120 (11.3%) had died (mean follow-up = 8.7 years, standard deviation (SD) = 3.3 years). Baseline post-bronchodilator FVC and FEV1/FVC were inversely associated with mortality. When both FVC and FEV1/FVC were mutually adjusted for each other, the decreased mortality rates were more pronounced for each standard deviation higher FVC at baseline (44% (95% confidence interval (CI): 25%, 58%) for men and 28% (95%CI: 11%, 41%) for women) than for FEV1/FVC at baseline (14% (95% CI: 8%, 20%) for men and 7% (95% -10%, 21%) for women). The probability of true regional differences was low.ConclusionsPeople with a higher FVC adjusted for age, sex and height have a longer survival. Regional adjustments to lung function standards are inappropriate when assessing prognosis.

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