Lung function follows a life-course trajectory, rising through childhood and adolescence to a peak in early adulthood; a low peak is associated with later COPD, cardiovascular morbidity and premature mortality. Childhood and adolescence are therefore the key periods during which adult respiratory reserve is built. Climate change is relevant to paediatric respiratory health not only because it triggers acute symptoms, but because it intensifies, prolongs and reschedules exposures already known to affect the developing lung, including heat, air pollution, wildfire smoke, aeroallergens and indoor damp. In this mini-review, we use lung-function trajectories as the organising framework and examine how climate-sensitive exposures may shift them downwards through three developmental pathways. First, early developmental disruption, including gestational air pollution and ambient temperature, may lower the starting point of the trajectory through fetal growth restriction, preterm birth and possibly direct effects on developing airways. Second, sustained exposure to air pollution, heat and indoor dampness across childhood and adolescence may impair postnatal lung growth and reduce lung-function growth and the peak attained. Third, climate-sensitive triggers increasingly precipitate exacerbations followed by incomplete recovery, particularly in asthma and cystic fibrosis, resulting in exacerbation-related step-down events. Crucially, these trajectories are not fixed; evidence that improving air quality accelerates lung-function growth shows that respiratory reserve can still be protected. We translate this trajectory-centred evidence into practical, climate-aware paediatric care, shifting the focus from short-term symptom prevention to preserving the lung-function reserve children carry into adulthood.
Background/Objective Chronic pulmonary aspergillosis (CPA) is a chronic progressive fungal lung infection associated with global mortality and impact on health-related quality of life (HRQoL). HRQoL tools are valuable in disease management and as trial end-points; however, use in CPA has not been systematically analysed. We analyse CPA-specific symptoms and strengths and limitations of currently used HRQoL tools focusing on psychometric validity. Methods A systematic review of studies reporting HRQoL tools or symptom scores in CPA and published in English (inception to May 2025) was conducted. Eligible studies were categorised into two groups, those employing HRQoL questionnaires (questionnaire-based studies (QBS) and those reporting CPA symptoms to analyse content validity. Narrative synthesis followed Synthesis Without Meta-analysis (SWiM) reporting guidance. Results Of 520 studies identified, 64 met inclusion criteria, including 18 QBS. Only generic tools have measured HRQoL in CPA, with St. George's Respiratory Questionnaire (SGRQ) the most common. SGRQ demonstrated excellent internal consistency (Cronbach's alpha 0.94), test–retest reliability (intraclass correlation coefficient 0.97) and an anchor-based minimal clinically important difference of 7.3 points; an >8-unit change in the SGRQ symptoms domain discriminated clinical recurrence at 12 months with 90.9% sensitivity and 87.0% specificity. Significant limitations however remain regarding content validity and usability. Other HRQoL tools or symptom scores used had minimal psychometric validity. Conclusion Current CPA HRQoL tools are generic with SGRQ the only tool with psychometric validity but limitations in content validity and usability highlighting the need for development of a CPA-specific HRQoL tool to aid clinical monitoring and standardisation of therapeutic end-points.
Obliterative bronchiolitis associated with connective tissue diseases (OB-CTD) is a severe and progressive pulmonary condition. Characterised by inflammation and fibrosis of the small airways, it leads to significant morbidity. Despite its severity, OB-CTD often remains underdiagnosed and the lack of standardised, evidence-based treatment guidelines poses a substantial clinical challenge. This scoping review consolidates current knowledge of the treatment of OB-CTD. The review aims to raise awareness and stimulate targeted research to improve patient outcomes. Current therapeutic regimens consist of empirical combinations of corticosteroids and various immunomodulatory agents, but their efficacy is inconsistent and supported by limited robust evidence. While supportive care, pulmonary rehabilitation and ultimately lung transplantation are key components of advanced disease management, this review highlights the critical need for effective, validated therapies. Emerging treatments, including antifibrotics, biologic therapies (targeting B-cells or tumour necrosis factor-α), Janus kinase inhibitors and phosphodiesterase-4B inhibitors, show promise but require rigorous investigation in the OB-CTD cohort. To advance the management of this challenging disease, future research must prioritise well-designed, large-scale studies on early diagnostic markers, distinct patient phenotypes, and novel therapeutic interventions.
Introduction Access to and engagement with asthma care could improve outcomes, but there is limited understanding of the reasons for poor engagement and a gap in actionable evidence to inform how to reduce inequities. We aimed to understand the context, mechanisms and outcomes (CMOs) that affect access to and engagement with asthma care and to identify solutions. Methods Using rapid realist review methodology, we searched four databases (1999–2024) for studies of any design in asthma populations (>5 years of age) that explored access or engagement in healthcare applicable to the UK health context. We extracted CMO configurations explaining access and engagement with asthma services and synthesised them into overarching theories. External reference group meetings with 16 experts with lived experience and professional expertise were held to discuss preliminary review findings and inform their interpretation. Results 19 articles reported 16 studies. We identified eight theories from 51 CMOs, grouped under two themes. 1) Demographic and social–cognitive barriers, including socioeconomic and structural barriers, uncertain candidacy resulting in escalation to emergency care, low perceived need hindering engagement and relational accountability driving engagement. 2) Health system barriers, including over-burdened services, fragmented systems disrupting care, feeling unheard hindering youth engagement, and cultural and language barriers. Solutions included digital care, education, developing patient skills, proactive follow-up, training for healthcare professionals, information sharing between services and enhanced professional–patient relationships. Conclusion Enhancing system coordination, cultural sensitivity and patient-centredness of care may improve trust and patient–provider communication, leading to better engagement and proactive asthma care.
The term “sarcoidosis”, coined in 1899 from the Greek sarx (flesh) and - oid (resembling), is descriptive and conveys no aetiological information. When a trigger has been identified for a granulomatous condition previously considered idiopathic, nomenclature has tended to shift from descriptive to aetiological, as when “Salem sarcoid” was reclassified as chronic beryllium disease (CBD). Crystalline silica exposure has been associated with sarcoidosis in several occupational cohorts, and partially overlapping immunopathogenic pathways have been described, notably NLR family pyrin domain containing 3 (NLRP3) inflammasome activation and T-helper cell 17.1 (Th17.1) polarisation, neither of which is disease-specific. The available evidence is consistent with a continuum model of silica-induced lung disease spanning a silicotic fibrotic phenotype (classical silicosis, driven by NLRP3 and transforming growth factor-β1 signalling), a granulomatous phenotype (silica-induced sarcoid-like reaction (SISLR), driven by NLRP3, interleukin-1β and Th17.1 signalling in susceptible individuals) and a mixed phenotype in which both processes coexist. The silica lymphocyte proliferation test (Si-LPT) has detected in vitro immunoreactivity in a subset of patients with sarcoidosis but is not standardised. The CBD paradigm offers useful analogies, although beryllium-driven adaptive and silica-driven innate mechanisms differ in important respects. SISLR may serve as a working classification that identifies the putative trigger, supports occupational disease recognition and situates the granulomatous response within a broader continuum; we discuss it alongside the alternative “silicosarcoidosis” terminology. On the evidence reviewed, crystalline silica should no longer be treated as an incidental co-exposure in a patient with sarcoid-type granulomas. A documented exposure warrants classification as occupationally related granulomatous lung disease and standardising the Si-LPT is the immediate priority.
