Current Opinion in Pulmonary Medicine最新文献

Purpose of review: Interstitial lung disease (ILD) comprises a diverse group of conditions characterized by lung inflammation and fibrosis. Cumulative lifetime exposures (i.e. the exposome) contribute to ILD onset and progression by interacting with genetic susceptibility and influencing multiple molecular pathways. This review summarizes current evidence evaluating how environmental exposures interact across the genome, epigenome, transcriptome, proteome, metabolome, and microbiome to drive ILD pathogenesis.

Recent findings: Environmental exposures, including air pollution, influence ILD risk through interactions with genetic factors that modify disease susceptibility. Epigenetic mechanisms, particularly DNA methylation, reflect key pathways through which exposures may contribute to ILD onset and progression and serve as sensitive biomarkers of environmental injury. Exposure-associated molecular alterations can be detected across multiple omic layers, including transcriptomic, proteomic, and metabolomic profiles. In parallel, exposures like cigarette smoking, silica, and air pollution influence the respiratory microbiome, with potential downstream effects on immune responses and fibrogenesis. Integrating these findings highlights environmentally-sensitive pathways that may represent novel targets for therapeutic modulation.

Summary: Integration of exposomic and multiomic molecular frameworks offers new opportunities to improve ILD risk stratification, prognostication, and precision therapeutic development, while also strengthening our mechanistic understanding of environmentally-mediated disease.

Purpose of review: Oxygen therapy is a key component of interstitial lung disease (ILD) management. This review summarizes the current evidence and explores how to balance its benefits and burdens in people with ILD.

Recent findings: Hypoxemia frequently occurs in ILD and is associated with worse prognosis, but the extent to which oxygen therapy modifies outcomes remains uncertain. Observational findings suggest home oxygen therapy may reduce acute exacerbations and hospitalizations in people with ILD with a life-expectancy of more than 1 year. Ambulatory oxygen can improve symptoms and health-related quality of life in some patients; however, recent evidence indicates that portable oxygen concentrators may not improve daily oxygenation, symptoms, or physical activity. In patients with isolated exertional or nocturnal desaturation or significant respiratory symptoms with hypoxemia, careful consideration of individual benefit versus burden is essential within a shared decision-making framework. High-flow oxygen therapy is effective for acute respiratory failure, but its role in palliative care, pulmonary rehabilitation, and especially in the home environment, are areas of ongoing investigation.

Summary: Oxygen therapy can support symptom relief in ILD, but its impact on long-term outcomes is unclear. High-quality evidence remains sparse, and advances in oxygen delivery technologies are needed to improve effectiveness while minimizing burden.

Purpose of review: This review aims to give an overview of recent advances in the clinical management of children <18 years of age with end-stage pulmonary hypertension refractory to conventional medical therapy.

Recent findings/summary: The increased awareness and characterization of pediatric pulmonary vascular disease has amounted more data toward creating an accurate, and comprehensive, risk prediction tool. This underscores the importance of serial outpatient re-assessment, as early consideration of advanced pharmaceutical and interventional therapies, as well as lung transplantation, improves outcomes in children. Despite the emergence and approval of new therapies against pulmonary hypertension in adults, pediatric-specific complications have not yet been adequately explored. It is, however, encouraging that several trials are ongoing to address this issue. Interventional strategies to unload the right ventricle are increasingly being utilized, although evidence remains scarce in this area. Extra-corporeal membrane oxygenation may, in addition to being a bridge to lung transplantation in well-selected patients, be considered as a bridge to catheter-based or surgical interventions. Management of pediatric right ventricular failure in the setting of advanced pulmonary hypertension is a truly complex clinical challenge at the intersection of cardiac, vascular and respiratory physiology and requires careful integration of invasive hemodynamic assessment, pulmonary vasodilator therapy, ventilatory strategy, and timely consideration of mechanical circulatory support.

Purpose of review: This review addresses the intersection between chronic pulmonary aspergillosis (CPA) and sarcoidosis, a clinically important but relatively understudied association. While few new sarcoidosis-specific data have emerged, recent advances in CPA research across broader underlying conditions (particularly structural lung diseases) provide updated insights into diagnosis, antifungal therapy, and management strategies that can be extrapolated to sarcoidosis.

Recent findings: CPA affects ~2% of sarcoidosis patients in tertiary cohorts and arises from combined structural lung damage, immune dysfunction, and environmental exposure. Diagnosis relies on integrated imaging, Aspergillus-specific immunoglobulin G, and new microbiological tools. Recent data refine therapeutic strategies regarding triazole selection, treatment duration, and salvage therapies. Management of haemoptysis relies on bronchial artery embolization, with emerging adjuncts such as local antifungal therapies and endobronchial valves. In addition, new epidemiological data have better characterized severe forms of CPA and refined estimates of mortality, highlighting a substantial disease burden. Serological markers may help predict relapse, and updated consensus criteria standardize response assessment.

Summary: CPA in sarcoidosis reflects advanced fibrocystic disease with complex host-pathogen interactions. Early recognition, optimized antifungal therapy, careful adjustment of immunosuppression, and structured haemoptysis management are central to care. Prospective sarcoidosis-specific studies are still needed to refine long-term strategies.

Purpose of review: This review describes recent developments in the phenotyping, monitoring, and treatment of advanced pulmonary sarcoidosis (APS), highlighting the positive strides made to recognise and treat this severe form of disease.

Recent findings: Key to this is the use of recently described high-resolution chest computed tomography (HRCT)-based phenotyping, the prognostic use of fibrosis extent coupled with pulmonary function tests, early echocardiography, and the increasing application of FDG-PET CT as a functional marker of inflammatory burden. Treatment paradigms have changed, with a shift from excessive corticosteroid use to steroid-sparing agents with less cumulative toxicity. Sarcoidosis-associated pulmonary hypertension (SAPH) is a well described lethal complication of APS with multiple potential underlying pathogenetic mechanisms. Directed therapies are dependent on the underlying mechanism but often vasodilators would be trialled given the multifactorial cause of the disease once SAPH has been confirmed.

Summary: APS is used to describe end-stage sequelae of pulmonary sarcoidosis, including established fibrotic lung disease with or without active inflammation and sarcoidosis-associated pulmonary hypertension. These manifestations are associated with significant morbidity and mortality, underscoring the need for early assessment, multidisciplinary involvement and ongoing vigilance.

Purpose of review: In pulmonary arterial hypertension (PAH), assessment of exercise provides important prognostic information. Recently, digital alternatives to traditional outcome measures, including digital versions of the six-minute walk test (6MWT), have been proposed. This review discusses existing methods of remote exercise assessment in PAH.

Recent findings: Summary metrics from actigraphy (e.g. daily steps) show promise on a population level but show high variability (e.g. related to seasonality) that may obscure clinically important changes. Conversely, digital structured exercise tests (6MWT) have proven safe, accurate compared to gold-standard tests and well-accepted by patients. Implementation and underlying algorithms vary, depending on whether tests are performed indoors or outdoors, along fixed or free courses, using accelerometery or GPS, and are delivered through app-only or app-and-wearable platforms. Integration of physiological data from wearables enhance digital 6MWT performance and hold promise as longitudinal endpoints. Key challenges include continued patient adherence and rigorous pre-processing of the raw data to ensure sustained data quality.

Summary: Future studies are needed to demonstrate the ability of the digital 6MWT or alternative exercise measures to reflect disease severity, show sensitivity to change and establish minimal clinically important differences for them to be implemented in clinical care for longitudinal monitoring.

Purpose of review: This review synthesizes the role of functional testing in the care of interstitial lung disease (ILD), evaluating the clinical utility of current approaches and discussing innovative strategies that may enhance patient monitoring and management.

Recent findings: The 6-min walk test (6MWT) remains the most studied and comprehensive assessment capturing functional and prognostic information, as well as outcomes related to interventions and treatments, in people with ILD. Step tests and sit-to-stand tests offer space-efficient alternatives with clinically relevant functional and prognostic insights. Current approaches are limited by accessibility and the ability to reflect patients' real-world performance. Advances in digital health and remote monitoring offer new opportunities. Physical activity trackers are widely available with step count and physical activity levels being associated with mortality, and are increasing used to evaluate effectiveness of pharmacological and nonpharmacological interventions. Smart home technologies is an emerging approach with potential for monitoring functional status, although evidence is limited.

Summary: Functional performance in people with ILD can be assessed using a range of approaches. With further research, remote functional testing, physical activity monitoring, and smart home technologies are poised to optimize the assessment of functional status in people with ILD.

Purpose of review: To discuss the most recent developments in quantitative imaging and artificial intelligence (AI) applications in interstitial lung diseases (ILD).

Recent findings: Aided by technical developments in the field, AI applications in chest imaging are increasingly being investigated, with recent algorithms showing improved performance compared with earlier techniques. This review article discusses the various roles of AI in fibrotic ILD, including diagnosis, characterization, quantification, and prognostication.

Summary: Increasing evidence supports the utility of quantitative CT and AI algorithms in improving visual assessment, increasing sensitivity and inter-observer agreement, as well as providing prognostic stratification in patients with a broad range of ILD. Nevertheless, the routine clinical application of these tools remains limited.

Purpose of review: Sarcoidosis is associated with an increasing global burden, driven by rising prevalence, severe organ involvement, treatment-related morbidity, and substantial impairment in quality of life (QoL). Despite advances in diagnostics and therapeutics, important barriers continue to limit timely, equitable, and patient-centered care. This review highlights three common challenges in sarcoidosis management and outlines pragmatic approaches to address them, using a patient-centered framework that emphasizes whether patients feel better, function better, and are able to thrive.

Recent findings: Recent evidence confirms persistent delays in diagnosis, limited access to sarcoidosis expertise, and inequities in referral to specialized care. Growing data indicates that chronic glucocorticoid use contributes substantially to long-term morbidity, supporting glucocorticoid stewardship and earlier use of steroid-sparing therapies. In parallel, patient-reported manifestations, such as fatigue, cognitive dysfunction, dysautonomia, and small fiber neuropathy-related symptoms, are increasingly recognized as major determinants of functional impairment and health-related QoL inadequately captured by traditional objective disease markers. Recent expert recommendations emphasize practical, implementable strategies applicable across both specialist and nonspecialist settings.

Summary: Improving sarcoidosis care requires a pragmatic, holistic approach that prioritizes timely diagnosis, equitable access to expertise, minimization of glucocorticoid-related harm, and systematic assessment of patient-reported outcomes. Collectively, these strategies support a patient-centered framework that evaluates treatment success not only by disease control but also by whether patients feel better, function better, and are able to thrive in the context of optimal health, meaningful social connection and contribution. This approach aligns care with patient priorities and supports individualized, shared decision-making.

Purpose of review: The diffusion capacity of the lungs for carbon monoxide ( DLCO ) is increasingly recognized as important diagnostic and prognostic marker in pulmonary hypertension. The review summarizes this diagnostic role and provides an overview of the clinical implications, potential underlying pathophysiology and impact of a decreased DLCO in different forms of pulmonary hypertension.

Recent findings: When in the diagnostic work-up of pulmonary arterial hypertension (PAH) a strongly lowered DLCO is found one must be aware of other causes and the diagnosis idiopathic PAH should be reconsidered. Left heart disease, lung disease, (peripheral) chronic thrombo-embolic pulmonary hypertension, underlying connective tissue disease and pulmonary veno-occlusive disease belong to the differential diagnosis. If all of these subforms of pulmonary hypertension are excluded and the patient fits the clinical profile the nowadays so called "Lung phenotype" should be considered. In general, a severely decreased DLCO is associated with a worse prognosis in different sub forms of pulmonary hypertension.

Summary: Reduced DLCO in pulmonary hypertension has important implications for clinical practice and research by improving diagnostic accuracy, supporting patient phenotyping, and identifying high-risk populations. Integration of DLCO into routine pulmonary hypertension assessment, registries, and future studies may enhance disease classification and facilitate more individualized diagnostic and therapeutic strategies.